Treatment for iris leiomyoma includes: Intraocular tumors

Description of the presentation Benign and malignant tumors of the iris and ciliary on slides

Benign and malignant tumors iris and ciliary body performed by: Kornienko L.A. Filshina I.A. Ivanova S.A.

CLASSIFICATION of benign tumors: Based on tissue affiliation, the following are distinguished: - myogenic tumors (leiomyoma); - epithelial (epithelioma): - vascular (hemangioma); - melanocytic tumors (nevus, melanocytoma).

Iris pigment epithelial adenoma is rare benign tumor in the form of a gray-black node with a bumpy surface, most often found on the periphery of the iris. The tumor leads to anterior displacement of the iris and thinning of its stroma, which atrophies over time, exposing the tumor, which is clearly visible during biomicroscopy.

Leiomyoma is an extremely rare benign tumor arising from smooth muscle. Clinically similar to non-pigmented melanoma, but unlike it, it is not always localized in the lower half of the iris. Often the diagnosis can only be made histologically. Expressiveness clinical manifestations depends on the degree of pigment saturation of the tumor. Pigmentless leiomyoma is represented by a yellowish-pink, translucent nodule of loose consistency, but with clear boundaries. Pigmented leiomyoma is light brown or dark brown in color. Based on the nature of growth, nodular, planar and mixed forms are distinguished. Most often localized in the ciliary belt of the iris.

A typical nevus is a pigmented, flat or slightly raised lesion, usually measuring less than 3 mm in diameter. It is located on the surface of the iris and can sometimes lead to deformation of the pupil and eversion of the pigment border. Freckles are smaller than nevi. They can be multiple and bilateral, but never violate the architecture of the iris.

Lisch nodules are multiple, small, bilateral, melanocytic hamartomas that occur after the age of 16 years in almost all patients with neurofibromatosis type I.

Hemangioma Congenital tumor. It can manifest itself in the first years of a child’s life and after 2-3 decades of life. Capillary, cavernous and racemosa hemangiomas can occur in the iris. Capillary hemangioma- tumor of newborns or children of the first years of life. On the iris it is presented in the form of intertwining small vessels dark color, growing into the tissue of the iris, occupying an area from one quadrant to half the surface of the iris. IN childhood such hemangiomas can regress on their own. As the process progresses, secondary glaucoma may develop. Cavernous hemangioma in the form of a node, it is often located at the pupillary edge, has a brownish-yellow or red color. The prominence of the tumor may vary depending on the degree of filling of its cavities with blood. Transient hyphemas are typical. Racemotic hemangioma in the iris is very rare in the form of sharply tortuous, intertwined vessels, located thicker than the iris and extending into the corner of the anterior chamber. Secondary glaucoma develops early.

IRIS CYSTS PRIMARY IRIS CYSTS These cysts are very rare and arise from the pigment epithelium or (less commonly) from the stroma. The vast majority are stationary and asymptomatic, especially those arising from the pigment epithelium. Epithelial cysts are unilateral, solitary, dark brown, round, translucent formations at the pupillary edge, in the middle zone or at the root of the iris. Sometimes the cyst may move and float freely in the anterior chamber or vitreous body. In most cases they are safe and treatment is rarely required.

Stromolic cysts are detected in the first year of life. Single, one-sided, with a smooth translucent front wall and liquid contents. The cyst can remain dormant for many years, and then suddenly enlarge and lead to secondary glaucoma and changes in the cornea. In this case, the fluid level in the cyst can simulate pseudohypopyon. Sometimes the cyst can break away from the iris and float in the anterior chamber or migrate to another location. There are cases of spontaneous regression of the cyst, but more often needle aspiration or surgical removal is performed.

Implantation cysts are generated by deposits epithelial cells from the surface of the conjunctiva or cornea into the iris after a penetrating wound OR surgical trauma, which can lead to the following formations. Serous cysts are clear, fluid-filled cysts and may be associated with a wound site. They usually increase in size, leading to corneal edema, anterior uveitis and glaucoma. Ultrasound biomicroscopy allows you to clarify the location and volume of the formation before surgery.

Malignant tumors of the iris Iris melanoma A malignant melanocytic tumor growing on the surface of the iris, often very slowly, arising from the stroma of the iris. It can spread to the angle of the anterior chamber, the anterior parts of the ciliary body.

Characterized by pigmented or non-pigmented node size<3 мм в диаметре и толщиной до 1 мм чаще в нижней половине радужки с ровной или неровной поверхностью. Выявляют поверхностную васкуляризацию опухоли, которую легче обнаружить у беспигментной, чем у интенсивно пигментированной опухоли, где сосуды не видны, а также деформацией зрачка, выворотом пигментной каймы, изредка встречается зонулярная катаракта. Признаки, связанные с эпителиоидно-клеточным компонентом, включают выраженную васкуляризацию, быстрый рост и неравномерную пигментацию.

TREATMENT - Observation of suspicious lesions consists of recording data from biomicroscopy, gonioscopy and photo registration. Monitoring must be lifelong as signs of growth may appear after a long period of dormancy. Initially, the examination is carried out every 3-6 months, then 6-9 months and then once a year. — Iridectomy is indicated for small tumors with iridoplasty to reduce postoperative photophobia. — Iridocyclectomy is performed for tumors growing into the angle of the anterior chamber. — Radiation therapy is carried out using applicators (brahmtherapy) or external irradiation of radiosensitive tumors. — Enucleation is indicated for diffusely growing tumors.

Metastasis A malignant, rapidly growing tumor in the iris that occurs as a result of hematogenous transfer of tumor cells from the main tumor node. Metastases to the iris reach just under 10% of the total number of intraocular metastases. Up to 47% of iris metastases in women are breast cancer, 25% in men are lung cancer.

Metastases to the iris are rare and are more often detected in patients with established metastatic disease. They resemble pink or yellowish, rapidly growing lesions that may be accompanied by anterior uveitis or, less commonly, hyphema. Small, multiple deposits may also be detected.

For melanoblastomas of the iris. Pigmented neoplasm of the iris spreads into the angle and covers its elements

Classification of ciliary body tumor: 1. Benign tumors: Fuchs adenoma Epithelioma Medulloepithelioma 2. Malignant tumors: Melanoma Metastatic tumors

Fuchs adenoma, or benign epithelioma, is extremely rare and, as a rule, is the subject of an accidental discovery. The tumor develops from non-pigmented ciliary epithelium and is represented by a round, light nodule, the size of which does not exceed 4 mm in diameter.

Epithelioma develops from the pigment ciliary epithelium and is somewhat more common. The tumor was detected at the age of 15-76 years, more often in women. There is a dark spot at the root of the iris or on the sclera at the limbus; dilated and tortuous episcleral vessels attract attention. More often, the tumor grows into the iris at the root, forming an intensely black, oval-shaped node. The edges are clearly defined, its surface is velvety.

Medulloepithelioma is rare. Refers to congenital neoplasms, is diagnosed more often in the first 4 years of a child’s life, there is information about the appearance of a tumor in adults. It grows slowly, locally it can acquire invasive growth, growing into the sclera. Growth is often accompanied by neovascularization of the iris according to the location of the tumor or neovascular glaucoma. More often, gray or pinkish tumor masses are found in the lower half of the ciliary body. Sometimes cysts on the surface of the tumor mask the tumor masses themselves. Secondary glaucoma may develop when the tumor grows in the basal zone of the iris and the angle of the anterior chamber. Young children may develop buphthalmos. Sometimes the tumor can become aggressive, destroy the eye and spread into the orbit.

Melanoma Isolated melanoma of the ciliary body accounts for less than 1% of all melanomas of the uveal tract. Occurs with equal frequency in men and women. The tumor grows slowly and can reach large sizes. A clearly defined, usually dark-colored, round-shaped tumor node. It has mixed localization: iridociliary or ciliochoroidal. They remain asymptomatic for a long time. Growing into the UPC is accompanied by the manifestation of iris folds located concentrically with the tumor, false iridodialysis. The shape of the pupil changes when the tumor grows into the pupil dilator. The edge of the pupil thickens, the pupil does not respond to light.

Amelanotic melanoma is pink in color and its own vessels are visualized. In the sector of tumor localization, congested, tortuous episcleral vessels are visible. Melanoma is accompanied by neovascularization of the iris and increased IOP. The tumor can lead to secondary glaucoma or retinal detachment.

Metastatic tumors Almost all human tumors metastasize to the ciliary body. Metastasis is characterized by the same symptoms as melanoma. The tumor is yellowish-white or pink in color, with a more rapid increase in symptoms. Secondary glaucoma and signs of anterior uveitis occur more often. Metastatic lesions of the iris and ciliary body are accompanied in 80% of cases by the development of Secondary glaucoma or iridocyclitis with “sebaceous” precipitates.

Diagnostics: Biomicroscopy Gonioscopy Microcycloscopy Transillumination Ultrasound MRI CT

Treatment - Monitoring of suspicious lesions consists of recording data from biomicroscopy, gonioscopy and photo registration. Monitoring must be lifelong as signs of growth may appear after a long period of dormancy. Initially, the examination is carried out every 3-6 months, then 6-9 months and then once a year. -For epitheliomas, organ-preserving treatment is indicated even when the tumor grows into the adjacent sclera. In such cases, the tumor is removed en bloc with the surrounding affected tissue. In such cases, we close the resulting defect with a sclerocorneal graft. In case of medulloepithalioma, taking into account the growth of the tumor, the tendency to destroy the sclera and grow into the orbit, it is advisable to perform enucleation. The prognosis for life is favorable.

For localized ciliary body melanomas, local excision may be used. Partial lamellar sclerouvectomy. Radiation treatment is possible. For large tumors occupying more than 1/3 of the circumference of the ciliary body, accompanied by a picture of nonspecific anterior uveitis or increased IOP, only enucleation is indicated. Treatment of metastatic tumors is palliative. External irradiation of the eye is possible. Enucleation is indicated for symptoms of painful glaucoma.

Leiomyoma of the iris develops from elements of the pupillary muscles, is characterized by extremely slow growth, and can be non-pigmented or pigmented. The variety of tumor colors can be explained by the morphogenesis of the iris. The fact is that the pupillary muscles of the iris are formed from the outer layer of the pigment epithelium of the iridociliary rudiment. During embryogenesis, iris sphincter cells produce melanin and myofibrils; in the postnatal period, the ability to produce melanin disappears, but the dilator retains it. This may explain the appearance of non-pigmented leiomyomas developing from the sphincter, and pigmented leiomyomas developing from dilator elements. The tumor is diagnosed mainly in the third - fourth decades of life.

Pigmentless leiomyoma of the iris grows locally in the form of a yellowish-pink translucent prominent node. The tumor is localized along the edge of the pupil or, less commonly, in the basal zone (in the area of ​​the ciliary crypts). The boundaries of the tumor are clear, the consistency is loose and gelatinous. On its surface, translucent outgrowths are visible, in the center of which there are vascular loops. Leiomyoma, located at the edge of the pupil, leads to eversion of the pigment border and a change in its shape. When the tumor is localized in the area of ​​the ciliary crypts, one of the first symptoms is an increase in intraocular pressure, so such patients are often diagnosed with unilateral primary glaucoma.

Pigmented leiomyoma of the iris is light to dark brown in color. The shape of the tumor can be nodular, flat or mixed. It is most often localized in the ciliary belt of the iris. Characteristic changes in the shape of the pupil, its elongation due to eversion of the pigment border directed towards the tumor. The consistency of pigmented leiomyoma is denser than that of non-pigmented leiomyoma, the surface is lumpy, and newly formed vessels are not visible. Tumor growth into the angle of the anterior chamber by more than 1/3 of its circumference leads to the development of secondary intraocular hypertension. Signs of tumor progression are considered to be changes around the tumor: smoothness of the iris relief and the appearance of a zone of pigment spraying, pigment tracks moving away from the tumor, a vascular rim in the iris; The shape of the pupil also changes. Growing into the structures of the anterior chamber angle and the ciliary body, the tumor extends into the posterior chamber, causing displacement and clouding of the lens. The diagnosis can be established based on the results of biomicro-, gonio-, diaphanoscopy and iridoangiography. Surgical treatment: the tumor is removed along with surrounding healthy tissue (block excision). If no more than 1/3 of the circumference of the iris is removed, its integrity can be restored by applying microsutures. As a result of restoring the integrity of the iris as a diaphragm, the severity and frequency of lens astigmatism are significantly reduced, and light aberrations are reduced. The prognosis for life is favorable; for vision, it depends on the initial size of the tumor: the smaller the tumor, the greater the likelihood of maintaining normal vision.

Nevus of the iris

The color of the iris and the pattern of the crypts are genetically programmed for each person. Its coloring is heterogeneous in almost everyone: areas of hyperpigmentation in the form of small spots - “freckles” - may be scattered across the surface of the iris. True nevi are classified as melanocytic tumors derived from the neural crest. They are detected in both children and adults. Clinically, an iris nevus looks like a more intensely pigmented area of ​​the iris. Its color varies from yellow to intense brown. The surface of the tumor is velvety, slightly uneven. Sometimes the nevus protrudes slightly above the surface of the iris. Its boundaries are clear, the pattern of the iris on the surface of the nevus is smoothed, in the central part, where the tumor is denser, there is no pattern. The size of the nevus ranges from an area of ​​pigmentation measuring 2-3 mm to large lesions occupying one quadrant of the iris surface or more. As the tumor progresses, it darkens and enlarges, a spray of previously unobserved pigment and a rim of dilated vessels appear around it, and the boundaries of the formation become less clear. Stationary nevi should be monitored. When the nevus progresses, its excision is indicated. The prognosis for life and vision is good.

We drink from the cup of existence With closed eyes, Wetting the golden edges with Our own tears.

M. Yu. Lermontov

In the 60-70s of the XX century. In ophthalmology, an independent clinical direction was identified - ophthalmo-oncology, which should resolve issues of diagnosis and treatment of tumors of the organ of vision. Tumors of this localization are characterized by great polymorphism and unique clinical and biological course. Diagnosing them is difficult; its implementation requires a complex of instrumental research methods, which are used by ophthalmologists. Along with this, knowledge about the application of diagnostic measures used in general oncology is required. Significant difficulties arise in the treatment of tumors of the eye, its adnexa and orbit, since a large number of anatomical structures important for vision are concentrated in small volumes of the eye and orbit, which complicates the implementation of therapeutic measures while maintaining visual functions.

The annual incidence of tumors of the organ of vision upon referral of patients is 100-120 per 1 million population. The incidence among children reaches 10-12% of the indicators established for the adult population. Taking into account the location, tumors of the adnexal apparatus of the eye (eyelids, conjunctiva), intraocular (choroid and retina) and orbit are distinguished. They differ in histogenesis, clinical course, professional and life

forecast. Among primary tumors, the first place in frequency is occupied by tumors of the adnexal apparatus of the eye, the second by intraocular tumors, and the third by orbital tumors.

20.1. Tumors of the eyelids

Benign tumors constitute the main group of eyelid tumors. The source of their growth can be skin elements (papilloma, senile wart, follicular keratosis, keratoacanthoma, senile keratosis, cutaneous horn, Bowen's epithelioma, xeroderma pigmentosum), hair follicles (Epithelioma of Malherbe, trichoepithelioma). Tumors originating from other tissues are less common.

Papilloma usually occurs after 60 years of age, its favorite localization is the lower eyelid. The tumor grows slowly and is characterized by

Rice. 20.1. Papilloma of the lower eyelid.

Rice. 20.2. Senile wart.

papillary growths of a spherical or cylindrical shape (Fig. 20.1). The color of the papilloma is grayish-yellow with a dirty coating due to the horny plates covering the surface of the papillae. The tumor grows from skin elements and has a developed stroma. The cellular elements are well differentiated, the covering epithelium is thickened. Treatment is surgical. Malignancy is observed in 1% of cases.

Senile wart develops after 50 years. It is localized in the temple area, eyelids, along the ciliary margin or in the intermarginal space, most often in the lower eyelid. It looks like a flat or slightly protruding formation with clear and even boundaries (Fig. 20.2). The color is gray, yellow or brown, the surface is dry and rough, the horny plates are differentiated. Growth is slow. Laser evaporation or cryodestruction are effective in treatment. There are known cases of malignancy, but without metastasis.

Senile keratosis appears after 60-65 years. It grows in areas exposed to insolation, especially often in the skin of the eyelids, in the form of multiple flat white areas covered with scales. Microscopic examination reveals thinning or atrophy of the epidermis. The most effective methods of treatment

treatments - cryodestruction and laser evaporation. If left untreated, malignancy occurs in approximately 20% of cases.

Cutaneous horn It is a finger-shaped skin outgrowth with elements of keratinization, its surface has a grayish-dirty tint. Diagnosed in elderly people. Electrical or laser excision is used in treatment.

Bowen's epithelioma represented by a flat, rounded spot of dark red color. The thickness of the tumor is insignificant, the edges are smooth and clear. It is covered with delicate scales, which, when removed, reveal a wet surface. Infiltrative growth appears during the transition to cancer. Effective methods of treatment are cryodestruction, laser evaporation and short-distance radiotherapy.

Xeroderma pigmentosum - a rarely observed disease with an autosomal recessive mode of inheritance. It manifests itself in young children (under 2 years old) in the form of increased sensitivity to ultraviolet radiation. In places exposed to even short-term insolation, foci of skin erythema appear, which are subsequently replaced by areas of pigmentation. The skin gradually becomes dry, thinned, rough, and telangiectasia develops in its atrophied areas. After 20 years, multiple tumor foci appear on the changed areas of the skin, along the edge of the eyelids, most often basal cell carcinoma. Treatment is to exclude ultraviolet radiation.

Capillary hemangioma in 1/3 of cases it is congenital, more often observed in girls. In the first 6 months of life, the tumor grows rapidly, then a period of stabilization begins, and by 7 years, in most patients, complete regression of the hemangioma is possible. The tumor has the appearance of brightly colored nodes

red or bluish. Most often localized on the upper eyelid, it grows, which leads to the appearance of partial and sometimes complete ptosis. As a result of the closure of the palpebral fissure, amblyopia develops, and due to the pressure of the thickened eyelid on the eye, corneal astigmatism occurs. There is a tendency for the tumor to spread beyond the skin of the eyelids. Microscopically, hemangioma is represented by capillary slits and stems filled with blood. Treatment of flat superficial capillary hemangioma is carried out using cryodestruction. For nodular forms, submersible diathermocoagulation with a needle electrode is effective; for common forms, radiation therapy is used.

Nevi - pigmented tumors - detected in newborns with a frequency of 1 case in 40 children; in the second and third decades of life, their number increases sharply, and by the age of 50 it decreases significantly. The source of nevus growth can be epidermal or dendritic melanocytes, nevus cells (nevocytes), dermal or spindle-shaped melanocytes. The first two types of cells are located in the epidermis, and the latter are located in the subepithelial layer. The following types of nevi have been identified.

Border(functional) nevus is characteristic of childhood, represented by a small flat dark spot, located mainly along the intermarginal edge of the eyelid. Treatment consists of complete electroexcision of the tumor.

Juvenile(spindle cell) nevus appears in children and young people in the form of a pinkish-orange well-circumscribed nodule, on the surface of which there is no hair. The tumor grows quite slowly. Treatment is surgical.

Rice. 20.3. Giant nevus of the skin of the eyelids.

Giant(systemic melanocytic). As a rule, the tumor is intensely pigmented, can be located on symmetrical areas of the eyelids, as it develops as a result of the migration of melanocytes at the stage of embryonic eyelids before their separation, covers the entire thickness of the eyelids, spreading to the intermarginal space, sometimes to the conjunctiva of the eyelids. The borders of the nevus are uneven, the color is light brown or intense black(Fig. 20.3). The tumor may have hair and papillary growths on the surface. Growth throughout the entire thickness of the eyelid leads to the appearance of ptosis. Papillary growths along the edge of the eyelids and abnormal growth of eyelashes cause lacrimation and persistent conjunctivitis. Treatment is effective with step-by-step laser vaporization, starting in infancy. The risk of malignancy with large nevi reaches 5%; foci of malignancy are formed in the deep layers of the dermis, and therefore its early diagnosis is almost impossible.

Nevus Ota, or oculodermal melanosis, arises from dermal melanocytes. The tumor is congenital, almost always one-sided,

manifests itself as flat spots of a reddish or purple color, usually located along the branches of the trigeminal nerve. Nevus of Ota may be accompanied by melanosis of the conjunctiva, sclera and choroid. Cases of malignancy have been described when Nevus of Ota is combined with uveal melanosis.

Benign nevi can progress at different rates and rates. In this regard, it is extremely important to highlight signs of nevus progression: the nature of pigmentation changes, a halo of delicate pigment forms around the nevus, the surface of the nevus becomes uneven (papillomatous), congestive blood vessels appear along the periphery of the nevus, and its size increases.

Malignant tumors eyelids are represented mainly by skin cancer and meibomian gland adenocarcinoma. Excessive ultraviolet radiation, especially in people with sensitive skin, the presence of non-healing ulcerative lesions or the influence of the human papillomatous virus play a role in development.

Basal cell carcinoma. The favorite localization of the tumor is the lower eyelid and the internal commissure of the eyelids. There are nodular, corrosive-ulcerative and scleroderma-like forms of cancer. Clinical signs depend on the shape of the tumor. With a nodular form, the boundaries of the tumor are quite clear (Fig. 20.4, a); it grows over the years, as its size increases, a crater-shaped recess appears in the center of the node, sometimes covered with a dry or bloody crust, after removing which a weeping, painless surface is exposed; the edges of the ulcer are callous. In the corrosive-ulcerative form, a small, almost imperceptible, painless ulcer with raised, shaft-like edges first appears. Gradually the area of ​​the ulcer increases, it

Rice. 20.4.Basal cell carcinoma. a - upper eyelid (nodal form); b - upper and lower eyelids (collapsing form).

covered with a dry or bloody crust and bleeds easily. After removing the crust, a rough defect is exposed, along the edges of which tuberous growths are visible. The ulcer is most often localized near the marginal edge of the eyelid, covering its entire thickness (Fig. 20.4, b). The scleroderma-like form in the initial stage is represented by erythema with a weeping surface, covered with yellowish scales. As the tumor grows, the central part of the weeping surface is replaced by a fairly dense whitish scar, and the progressive edge spreads to healthy tissue.

Rice. 20.5. Squamous cell carcinoma of the lower eyelid.

Squamous cell carcinoma. In the initial stage, the tumor is represented by mild erythema of the skin, most often of the lower eyelid. Gradually, a compaction with hyperkeratosis on the surface appears in the erythema zone. Perifocal dermatitis occurs around the tumor, and conjunctivitis develops (Fig. 20.5). The tumor grows over 1-2 years. Gradually, a depression with an ulcerated surface forms in the center of the node, the area of ​​which gradually increases. The edges of the ulcer are dense and lumpy. When localized at the edge of the eyelids, the tumor quickly spreads into the orbit.

Treatment of eyelid cancer is planned after receiving the results of a histological examination of the material obtained from a tumor biopsy. Surgical treatment is possible if the tumor diameter is no more than 10 mm. The use of microsurgical equipment, laser or radiosurgical scalpel increases the effectiveness of treatment. Contact radiation therapy (brachytherapy) or cryodestruction may be performed. When localizing

tumors near the intermarginal space can only be treated with external irradiation or photodynamic therapy. If the tumor grows onto the conjunctiva or into the orbit, subperiosteal exenteration of the latter is indicated. With timely treatment, 95% of patients live more than 5 years.

Meibomian gland adenocarcinoma (glands of the cartilage of the eyelid). The tumor is located under the skin, usually of the upper eyelid, in the form of a node with a yellowish tint, reminiscent of a chalazion, which recurs after removal or begins to grow aggressively after drug treatment and physical therapy. After removal of the chalazion, a histological examination of the capsule must be performed. Adenocarcinoma can manifest as blepharoconjunctivitis and meibomyitis, grows quickly, spreads to cartilage, palpebral conjunctiva and its fornix, lacrimal ducts and nasal cavity. Given the aggressive nature of tumor growth, surgical treatment is not indicated. For small tumors limited to the tissues of the eyelids, external irradiation can be used. If metastases appear in regional lymph nodes (parotid, submandibular), they should be irradiated. The presence of signs of tumor spread to the conjunctiva and its fornix necessitates orbital exenteration. The tumor is extremely malignant. Within 2-10 years after radiation therapy or surgical treatment, relapses occur in 90% of patients. 50-67% of patients die from distant metastases within 5 years.

Melanoma develops from transformed intradermal melanocytes. Risk factors for the development of melanoma: nevi, especially borderline ones, melanosis, individual increased sensitivity to intense solar radiation.

The clinical picture is polysymptomatic. Melanoma can be represented by a flat lesion with uneven and indistinct edges of a light brown color, with patchy, more intense pigmentation along the surface. The nodular form of melanoma (more often observed when localized on the skin of the eyelids) is characterized by a noticeable prominence above the surface of the skin; there is no skin pattern in this area. The tumor grows rapidly, its surface easily ulcerates, and spontaneous bleeding is observed. Even with the lightest touch of a gauze pad or cotton swab to the surface of such a tumor, a dark pigment remains on them (Fig. 20.6). The skin around the tumor is hyperemic as a result of dilation of the perifocal vessels, and a rim of sprayed pigment is visible. Melanoma spreads early to the mucous membrane of the eyelids, the lacrimal caruncle, the conjunctiva and its fornix, and into the orbital tissue. The tumor metastasizes to regional lymph nodes, skin, liver and lungs.

Treatment should be planned only after a complete examination of the patient to identify metastases. For melanomas with a maximum diameter of less than 10 mm and the absence of metastases, surgical excision can be performed using a laser scalpel, radio scalpel or electric knife with mandatory cryofixation of the tumor. Removal of the lesion is through, at a distance of at least 3 mm from the visible (under an operating microscope) boundaries. Cryodestruction for melanomas is contraindicated. Nodular tumors with a diameter of more than 15 mm with a rim of dilated vessels are not subject to local excision, since in this phase, as a rule, metastases are already observed. Radiation therapy using a narrow medical proton beam is an alternative

Rice. 20.6. Melanoma of the skin of the lower

century.

howling exenteration of the orbit. Regional lymph nodes should also be irradiated.

The prognosis for life is very difficult and depends on the depth of tumor spread. With the nodular form, the prognosis is worse, since vertical invasion of tissues by tumor cells occurs early. The prognosis worsens when melanoma spreads to the costal margin of the eyelid, intermarginal space and conjunctiva.

20.2. Tumors of the conjunctiva and cornea

Tumors of the conjunctiva and cornea are considered together, since the corneal epithelium is topographically a continuation of the conjunctival epithelium. The rich connective tissue base of the conjunctiva predisposes to the development of a wide range of tumors. Benign tumors (dermoids, dermolipomas, pigmented tumors) predominate in the conjunctiva and cornea, and in childhood they account for more than 99% of all tumors in this location.

Benign tumors. Dermoid refers to developmental defects. This is a whitish-yellow formation

Rice. 20.7. Tumors of the conjunctiva and cornea.

a - corneal dermoid; b - dermolipoma of the conjunctiva and cornea.

color, often located near the outer or lower outer limbus. With this localization, the tumor spreads early to the cornea and can grow to its deep layers. Dilated vessels approach the neoplasm. The surface of the dermoid on the cornea is smooth, shiny, and white. Microscopic examination reveals elements of sweat glands, fat lobules, and hair in the formation. Dermolipoma (Fig. 20.7) - a dermoid with a high content of adipose tissue, most often localized

in the area of ​​the conjunctival fornix. Treatment is surgical.

Papilloma. Tumor first type observed in children; it manifests itself as multiple nodules, most often localized on the lower fornix of the conjunctiva. The nodules are translucent with a smooth surface, consisting of individual lobules penetrated by their own vessels, which gives them a reddish-pink color. The soft consistency and thin stem-shaped base makes the nodules mobile and easily wounded: their surface bleeds even when lightly touched with a glass rod. In older patients, keratinizing papilloma (second type), as a rule, it is localized near the limbus in the form of a single immobile formation of a grayish-white color. Its surface is rough, the lobules are poorly distinguishable. With this localization, the papilloma spreads to the cornea, where it looks like a translucent formation with a grayish tint. Papilloma of the first type is microscopically represented by non-keratinizing papillary growths, in the center of which vascular loops are located. Such papillomas can spontaneously regress. Given the multifocal nature of the lesions, their surgical treatment is often ineffective; laser evaporation or application of a 0.04% mitomycin C solution to the affected area is indicated. Keratinizing papilloma (second type) is characterized by papillary hyperplasia of the epithelium with pronounced phenomena of para- and hyperkeratosis. Such a papilloma is subject to laser excision, as cases of its malignancy have been described. If the tumor is completely removed, the prognosis is good.

Bowen's epithelioma is a flat or slightly protruding plaque above the surface of the conjunctiva with clear gray borders; with pronounced vascularization it may have a reddish color

shade. Bowen's epithelioma occurs in the epithelium and can penetrate into the deep layers of the conjunctiva, but the basement membrane always remains intact. Spreading to the cornea, the tumor does not invade Bowman's membrane. Treatment is surgical or combined, including treatment of the tumor with a 0.04% solution of mitomycin C 2-3 days before surgery, excision of the tumor and treatment of the surgical wound with a solution of mitomycin C on the operating table and in the next 2-3 days. Short-distance radiotherapy is effective.

Vascular tumors conjunctivae are represented by capillary hemangioma and lymphangioma; belong to the group of hamartomas, are observed from birth or appear in the first months of life. Capillary hemangioma most often localized in the inner corner of the palpebral fissure, it consists of sharply tortuous cyanotic vessels of small caliber that infiltrate the semilunar fold and the conjunctiva of the eyeball. Spreading to the fornix, the vessels can penetrate into the orbit. Spontaneous hemorrhages are possible. Treatment consists of dosed submersible electrocoagulation. At an early stage, laser coagulation is effective.

Lymphangioma Hemangiomas are much less common and are represented by dilated thin-walled vascular channels of irregular shape, the inner surface of which is lined with endothelium. These channels contain serous fluid mixed with red blood cells. The tumor is localized in the conjunctiva of the eyeball or its fornix. The process involves the semilunar fold and the lacrimal caruncle. The tumor looks like a translucent yellowish thickening of the conjunctiva, consists of small lobules filled with clear liquid, sometimes mixed with blood. On the surface of lymphangioma there are often visible small

some hemorrhages. In the lobules and between them there are blood-filled vessels. The tumor infiltrates the soft tissues of the orbit. Small, non-spread lymphangiomas can be treated with a CO 2 laser. For more widespread tumors, brachytherapy using a strontium applicator with removal of the cornea from the irradiation zone can be recommended.

Nevus - pigment tumor. According to the clinical course, nevi are divided into stationary and progressive, blue nevus and primary acquired melanosis.

Stationary nevus. The favorite localization is the conjunctiva of the eyeball in the area of ​​the palpebral fissure; it never occurs in the mucous membrane of the eyelids. The color of the nevus is from light yellow or pink to light brown with a well-developed vascular network. Typically the tumor is located near the limbus. Up to 1/3 of stationary nevi are pigmentless. During puberty, the color of the nevus may change. The surface of the tumor is smooth or slightly rough due to the formation of small light cysts in it, the boundaries are clear. When localized in the conjunctiva of the eyeball, nevi easily move above the sclera, but at the limbus they are motionless (Fig. 20.8). semilunar fold

Rice. 20.8. Nevus of the lacrimal caruncle.

The nevus is thickened, and in the area of ​​the lacrimal caruncle the tumor is slightly prominence. Its boundaries are clear.

For progressive nevus characterized by an increase in size and color change. The surface of the nevus looks motley: along with non-pigmented or weakly pigmented areas, zones of intense pigmentation appear, the boundaries of the tumor become less clear due to the spraying of pigment. Accumulation of pigment can also be observed outside the visible boundaries of the tumor. The tumor's own vessels dilate significantly and their number increases. The presence of a triad of signs - increased pigmentation, vascularization of the nevus and blurred borders- allows you to differentiate the true progression of the tumor from its increase due to reactive epithelial hyperplasia. Limitation of the displacement of the nevus in relation to the sclera is a late symptom indicating the development of melanoma. Treatment - excision of the nevus - is indicated when signs of its growth appear.

Blue (cellular) nevus- congenital formation, which is extremely rare. It is considered as one of the symptoms of systemic skin damage in the oculodermal area. The formation is flat, reaches large sizes, does not have a clear shape, but its boundaries are well defined. Blue nevus can be combined with melanosis. Treatment is not required, since malignant variants of blue nevus in the conjunctiva have not been described.

Primary acquired melanosis(PPM) of the conjunctiva is usually unilateral. PPM lesions are flat, with fairly clear boundaries, and have an intensely dark color. Reaching the limbus, the tumor easily spreads to the cornea. Treatment consists of extensive laser coagulation or electroexcision of the tumor with preliminary

Rice. 20.9. Melanoma of the conjunctiva and eyelid against the background of primary acquired melanosis of the conjunctiva.

solid applications of a 0.04% solution of mitomycin C. With a small spread of PPM, cryodestruction gives good results. In cases of damage to the fornix and tarsal conjunctiva, brachytherapy is more effective. The prognosis is unfavorable, since in 2/3 of cases the PPM undergoes malignancy (Fig. 20.9).

Malignant tumors. Squamous cell carcinoma rarely observed. Provoking factors include ultraviolet radiation, human papillomatous virus and HIV infection. The tumor may have the appearance of a papillomatous whitish-pink node and even a whitish pterygium in combination with elements of inflammation. Its boundaries are unclear; chaotically located delicate native vessels are clearly visible on the surface of the tumor papillae. The tumor is characterized by rather slow growth. Its aggressiveness is due to invasion into deeper tissues, destruction of the cornea, sclera and germination of tumor masses into the eye cavity. The choice of treatment method is determined by the location and size of the tumor. For small tumors located on the limbus and cornea, instillation of mitomi-

cin C according to a special scheme for 2 weeks. A combination of local excision of the tumor with cryodestruction is possible. When the tumor is localized outside the limbus and cornea, brachytherapy in combination with local laser coagulation or electrical excision or wide excision with simultaneous cryoapplications along the wound surface are indicated.

Melanoma conjunctiva develops from primary acquired melanosis and pre-existing nevi or is primary. The tumor can be pigmented or non-pigmented, the latter being asymptomatic for a long time; grows quickly in the form of a node or superficially; sometimes multiple foci are formed, which can merge (Fig. 20.10). The surface of melanoma is smooth and shiny. In the pigmented form, radially located pigment “tracks” or a scattering of pigment are visible at the border of the node. A network of dilated, stagnant, full-blooded vessels is formed around the tumor. As melanoma grows, its surface becomes ulcerated and the tumor begins to bleed. The appearance of satellites is characteristic as a result of the formation of screenings and contact with the main tumor node. Pigmentless screenings are especially dangerous, since due to their pink color the doctor often does not notice them. Melanoma often grows into the cornea.

Treatment of the tumor should begin as early as possible. For localized melanoma, combined organ-preserving treatment is indicated; local excision and brachytherapy, local chemotherapy with mitomycin C and local block excision (removal of the tumor with surrounding healthy tissue) can be performed. For widespread tumors, as well as for melanoma of the lacrimal caruncle and semilunar fold, treatment is effective.

Rice. 20.10. Melanoma of the conjunctiva.

irradiation with a narrow medical proton beam.

The prognosis for conjunctival melanoma is poor. With hematogenous metastasis, mortality reaches 22-30%. With adequate treatment, the 5-year survival rate is 95%. The outcome of treatment largely depends on the location and size of the tumor. For melanomas up to 1.5 mm thick, the prognosis is better. If the tumor thickness reaches 2 mm or more, the risk of regional and distant metastases increases. The prognosis worsens if the tumor spreads to the lacrimal caruncle, fornix and palpebral conjunctiva. With epibulbar melanoma, especially localized in the limbus, the prognosis is more favorable.

20.3. Intraocular tumors

Among neoplasms of the organ of vision, intraocular tumors occupy the second place in frequency, most of them are malignant.

20.3.1. Tumors of the choroid

Tumors of the choroid are often represented by tumors of neuroectodermal origin, less often - of mesodermal origin.

20.3.1.1. Iris tumors

Up to 84% of iris tumors are benign, more than half of them are myogenic in nature.

Benign tumors. Leiomyoma develops from elements of the muscles of the iris, is characterized by extremely slow growth, and can be pigmentless or pigmented.

Amethymic leiomyoma grows locally in the form of a yellowish-pink translucent prominent node (Fig. 20.11, a). The tumor is localized along the edge of the pupil or, less commonly, in the basal zone (in the area of ​​the ciliary crypts). The boundaries of the tumor are clear, the consistency is loose and gelatinous. On its surface, translucent outgrowths are visible, in the center of which there are vascular loops. Leiomyoma, located at the edge of the pupil, leads to eversion of the pigment border and a change in its shape. When the tumor is localized in the area of ​​the ciliary crypts, one of the first symptoms is an increase in intraocular pressure, so such patients often

A diagnosis of unilateral primary glaucoma is made.

Pigmented leiomyoma has a color ranging from light to dark brown. The shape of the tumor can be nodular, flat or mixed (Fig. 20.11, b). It is most often localized in the ciliary belt of the iris. Characteristic changes in the shape of the pupil, its elongation due to eversion of the pigment border directed towards the tumor. The consistency of pigmented leiomyoma is denser than that of non-pigmented leiomyoma, the surface is lumpy, and newly formed vessels are not visible. Tumor growth into the angle of the anterior chamber by more than 1/3 of its circumference leads to the development of secondary intraocular hypertension. Signs of tumor progression are considered to be changes around the tumor: smoothness of the iris relief and the appearance of a zone of pigment spraying, pigment tracks moving away from the tumor, a vascular rim in the iris; The shape of the pupil also changes. Growing into the structures of the anterior chamber angle and the ciliary body,

Rice. 20.11. Leiomyoma of the iris. a - pigmentless; b - pigment.

Rice. 20.12. Iridoangiogram. Leiomyoma of the iris. Newly formed tumor vessels are clearly contrasted.

the tumor extends into the posterior chamber, causing displacement and clouding of the lens. The diagnosis can be established based on the results of biomicro-, gonio-, diaphanoscopy and iridoangiography (Fig. 20.12). Surgical treatment: the tumor is removed along with surrounding healthy tissue (block excision). The prognosis for life is favorable; for vision, it depends on the initial size of the tumor: the smaller the tumor, the greater the likelihood of maintaining normal vision.

Nevus. Clinically, a nevus looks like a more intensely pigmented area of ​​the iris. Its color varies from yellow to intense brown. The surface of the tumor is velvety, uneven. Sometimes the nevus protrudes slightly above the surface of the iris. Its borders are clear, the pattern of the iris on the surface of the nevus is smoothed, in the central part, where the tumor is denser, there is no pattern. The size of the nevus ranges from an area of ​​pigmentation measuring 2-3 mm to large lesions occupying one quadrant of the iris surface or more. When pro-

As the tumor progresses, it darkens and enlarges; a spray of previously unobserved pigment and a rim of dilated vessels appear around it; the boundaries of the formation become less clear. Stationary nevi should be monitored. When the nevus progresses, its excision is indicated. The prognosis for life and vision is good.

Malignant tumors. Melanoma. According to the growth pattern of melanoma, there are nodular, diffuse (extremely rare) and mixed. Nodular melanoma has the appearance of a delimited node protruding into the anterior chamber. The surface of the tumor is uneven, the depth of the anterior chamber is uneven. The color of melanoma varies from light to dark brown. Growing into the stroma of the iris, the tumor can imitate a cyst. When melanoma comes into contact with the posterior epithelium of the cornea, local opacification occurs. Growing through the iris dilator, the tumor leads to a change in the shape of the pupil: its edge on the side of the tumor becomes flattened and does not respond to mydriatics. The tumor can fill the posterior chamber, causing lens compression, opacification, and posterior dislocation. Complexes of tumor cells are scattered over the surface of the iris, and it takes on a variegated appearance (Fig. 20.13). Due to the germination

Rice. 20.13. Iris melanoma (iridociliary localization).

When the tumor moves into the angle of the anterior chamber, the outflow of intraocular fluid is disrupted, and persistent intraocular hypertension, which is not amenable to drug therapy, develops.

The diagnosis is established based on the results of biomicro-, gonio-, diaphanoscopy and fluorescent iridoangiography. Treatment is surgical. Localized melanoma of the iris, occupying no more than 1/3 of its circumference, is subject to local removal. Local photodynamic therapy is possible. If the lesion is larger, enucleation of the eyeball should be recommended. The prognosis for life is usually favorable, given the predominance of the spindle cell type of tumor. Metastasis is observed in 5-15% of cases and mainly with large tumors. The prognosis for vision after organ-preserving operations is usually favorable.

20.3.1.2. Tumors of the ciliary body

Benign tumors are rarely observed, represented by adenoma, epithelioma, medulloepithelioma.

Malignant tumors occur more often.

Melanoma ciliary body. According to the morphological characteristics, this tumor is no different from melanomas of the choroid and iris, but there is a predominance of its epithelioid and mixed forms. Through the wide pupil, a clearly demarcated node of a round shape, often dark in color, is clearly visible. For a long period of time, the disease is asymptomatic. When a tumor grows into the dilator, the shape of the pupil changes. The pupil does not react to light. Amelanotic melanoma has a pinkish tint and its own vessels are well visualized.

In the sector where the tumor is localized, congested, tortuous episcleral vessels are visible. In later stages, secondary glaucoma develops. The tumor can grow into the sclera, forming a node under the conjunctiva, often dark in color.

Biomicroscopy, microcycloscopy with a wide pupil, gonio- and diaphanoscopy help in diagnosing melanoma of the ciliary body. When treating localized melanomas of the ciliary body, one can limit oneself to its removal (partial lamellar sclerouvectomy). Radiation treatment is possible. For large tumors (occupying more than 1/3 of the circumference of the ciliary body), only enucleation of the eyeball is indicated. Tumor growth of the scleral capsule with the formation of subconjunctival nodes necessitates enucleation in the absence of regional or hematogenous metastases that have been instrumentally proven. The prognosis depends on the cellular composition and size of the tumor.

20.3.1.3. Choroidal tumors

Benign tumors occur rarely and are represented by hemangioma, osteoma and fibrous histiocytoma.

Hemangioma - a rare congenital tumor, classified as hamartoma. It is discovered accidentally or due to visual impairment. Decreased vision is one of the early symptoms of a growing hemangioma in adults; in childhood, strabismus primarily attracts attention. In the choroid, a hemangioma almost always has the appearance of an isolated node with fairly clear boundaries, round or oval in shape, the maximum tumor diameter is 3-15 mm. The prominence of the tumor varies from 1 to 6 mm. Usually the color of the tumor is pale gray or yellowish-pink, it can be intense

deep red. Secondary retinal detachment and its folding are observed in almost all patients; with large tumors it becomes vesicular.

Diagnosis of cavernous hemangioma is difficult. Discrepancies between clinical data and histological examination results are observed in 18.5% of cases. In recent years, diagnosis has become more reliable through the use of fluorescein angiography. Treatment was considered futile for a long time. Currently, laser coagulation or brachytherapy (contact irradiation using radioactive ophthalmic applicators) is used. The prognosis for life is favorable.

Pigmented tumors. Nevus- the most common benign intraocular tumor. In the fundus they have the appearance of flat or slightly protruding foci (up to 1 mm in height) of light gray or gray-green color with feathery but clear boundaries, their diameter ranges from 1 to 6 mm (Fig. 20.14). Pigmentless nevi have an oval or round shape, their borders are smoother, but less clear due to the lack of pigment.

Patients with stationary nevi do not require treatment, but they do need clinical observation, since during the course of life the tumors can grow, moving into the stage of a progressive nevus and even initial melanoma. Progressive nevi, taking into account the potential for their degeneration and transition to melanoma, are subject to treatment. The most effective method of destroying a progressive nevus is laser coagulation.

Melanoma- large cell nevus with a benign course, usually localized on the optic nerve head, but can also be located in other parts of the joint;

Rice. 20.14. Choroidal nevus.

choroid. The tumor is asymptomatic, and in 90% of patients it is detected incidentally. With large melanocytomas, slight visual disturbances and an enlarged blind spot may be observed. The color of the tumor in most patients is intense black; there may be light spots on the surface - drusen of the vitreous plate (Fig. 20.15). Treatment

Rice. 20.15. Choroidal melanocytoma, localized in the peripapillary region and extending to the optic nerve head.

Rice. 20.16. Choroidal melanoma.

a - fundus of the eye; b - gross specimen of an eye with choroidal melanoma.

not required, but patients should be under systematic observation by an ophthalmologist.

Malignant tumors presented mainly melanomas. Spindle cell melanoma metastasizes in almost 15% of cases. The frequency of metastasis of epithelioid melanoma reaches 46.7%. Thus, cellular

Rice. 20.17. Pigmentless nodule of choroidal melanoma with hemorrhages on the surface of the tumor.

The characteristics of uveal melanoma are one of the determining factors of prognosis for life. More than half of melanomas are located beyond the equator. The tumor, as a rule, grows in the form of a solitary node (Fig. 20.16). Typically, patients complain of blurred vision, photo- and morphopsia.

In the initial stage, the tumor is represented by a small lesion of brown or dark gray color. As the tumor grows, its color may become more intense (sometimes even dark brown) or it remains yellowish-pink, without pigment (Fig. 20.17). An increase in the thickness of melanoma causes dystrophic changes in Bruch's membrane and the retinal pigment epithelium, as a result of which the integrity of the vitreous plate is disrupted and the tumor grows under the retina - the so-called mushroom-shaped form of melanoma with a wide base is formed (Fig. 20.18).

It is extremely difficult to recognize choroidal melanoma in opaque media. In such cases, additional diagnostics help to clarify the diagnosis.

Rice. 20.18. Ultrasound scan of the eye. The shadow of a forming mushroom-shaped melanoma of the choroid and secondary retinal detachment are visible.

specific research methods (ultrasound scanning, computed tomography). Before deciding on the nature of treatment, the oncologist must conduct a thorough examination of the patient in order to exclude metastases. It should be remembered that upon initial contact with an ophthalmologist, metastases are detected in 2-6.5% of patients with large tumors and in 0.8% with small melanomas.

For more than 400 years, the only treatment for choroidal melanoma was enucleation of the eyeball. Since the 70s of the 20th century, organ-preserving treatment methods have begun to be used in clinical practice, the goal of which is to preserve the eye and visual functions subject to local destruction of the tumor. These methods include laser coagulation, hyperthermia,

cryodestruction, radiation therapy (brachytherapy and tumor irradiation with a narrow medical proton beam). Naturally, organ-preserving treatment is indicated only for small tumors.

Melanoma metastasizes hematogenously, most often to the liver (up to 85%), the second place in the frequency of occurrence of metastases is occupied by

there are lungs. The use of chemotherapy and immunotherapy for metastases of uveal melanoma is still limited due to the lack of a positive effect. The prognosis for vision after brachytherapy is determined by the size and location of the tumor. Vision can be preserved if the tumor is located outside the macular zone. The eye as a cosmetic organ is preserved in 80% of patients. After treatment, patients should be monitored almost until the end of their lives. After radiation therapy and local tumor removal, the doctor should examine patients every 3 months for the first 2 years, then 2 times a year during the 3rd and 4th years, then once a year.

20.3.1.4. Retinal tumors

Retinal tumors account for 1/3 of all intraocular neoplasms. Benign tumors(hemangioma, astrocytic hamartoma) are extremely rare. The main group is represented by the only malignant tumor retinas in children - retinoblastoma.

Predisposition to the occurrence of retinoblastoma is currently associated with the presence of a germline mutation in one of the alleles of the gene RB1, which is inherited in an autosomal dominant manner.

The tumor develops in young children (up to 1 year). In 2/3 of patients with a hereditary form of retinoblastoma, it turns out to be bilateral. In addition, in familial forms of retinoblastoma, the gene RB1 damaged in all somatic cells, so such patients have a high risk of developing tumors in other locations. The discovery of retinoblastoma in a child under 10 months of age indicates its congenital nature; a tumor after 30 months can be considered

be considered sporadic. The sporadic form accounts for about 60% of all retinoblastomas, always unilateral.

Retinoblastoma develops in any part of the optically active part of the retina, and at the beginning of its growth it looks like a violation of the clarity of the reflex in the fundus. Later, a grayish, cloudy, flat lesion with unclear contours appears. Subsequently, the clinical picture changes depending on the growth characteristics of retinoblastoma. There are endophytic, exophytic and mixed types of tumor growth.

Endophytically growing Retinoblastoma occurs in the inner layers of the retina and is characterized by growth into the vitreous. The surface of the tumor is lumpy. The thickness of the node gradually increases, the color remains whitish-yellow, the retinal vessels and the tumor's own vessels are not visible (Fig. 20.19). In the vitreous above the tumor, conglomerates of tumor cells appear in the form of stearin drops and stearin tracks. The rapid growth of the tumor, accompanied by disruption of metabolic processes in it, leads to the appearance of zones of necrosis with cheesy decay, subsequently calcification.

Rice. 20.19. Retinoblastoma (endophyte-

nal form).

associated with the formation of calcifications. When a tumor is localized in the preequatorial zone, its cells, settling in the posterior and anterior chambers of the eye, create a picture of a pseudohypopyon, the color of which, in contrast to the color of a true hypopyon, is whitish-gray. Increasing in size, the tumor fills the entire eye cavity, resulting in increased intraocular pressure. In young children, buphthalmos develops, thinning of the sclerolimbal zone occurs, which facilitates the spread of the tumor outside the eye. When a tumor grows into the sclera behind the equator, a picture of cellulite appears.

Exophytic growing Retinoblastoma occurs in the outer layers of the retina and spreads beneath the retina, resulting in a massive retinal detachment with a dome visible behind the clear lens. During ophthalmoscopy, the tumor is detected in the form of one or several delimited nodes with a smooth surface. Draining dilated and tortuous retinal vessels approach the tumor. On the surface of the tumor, delicate, tortuous, chaotically located newly formed vessels are visible.

Retinoblastoma is characterized by multifocal growth. Tumor nodes are localized in different parts of the fundus, have a round or oval shape, and their thickness varies. Sometimes hemorrhages on the surface of the tumor merge and completely cover the tumor. In such cases, with a peripheral location of retinoblastoma, the first symptom may be “spontaneously” occurring hemophthalmos.

Mixed retinoblastoma is characterized by a combination of ophthalmological symptoms characteristic of the two described forms. Well-known signs of retinoblastoma are pupillary glow and strabismus, heterochromia or rubeosis

irises, microphthalmos, buphthalmos, hyphema, hemophthalmos - should be regarded as indirect, which can be observed in other diseases. Retinoblastoma can occur without indirect signs and is usually discovered during routine examinations.

Retinoblastoma in older children is characterized by decreased visual acuity. The clinical picture is dominated by signs of sluggish uveitis, secondary painful glaucoma, retinal detachment, and retinal angiomatosis rarely develops. The occurrence of retinoblastoma at this age, when the likelihood of its development is low, complicates diagnosis.

Trilateral retinoblastoma is regarded as a bilateral tumor combined with an ectopic (but not metastatic!) intracranial tumor. The third tumor is usually localized in the pineal gland, but can also occupy the midline structures of the brain. Clinically, the tumor manifests itself 2-3 years after the discovery of bilateral retinoblastoma.

Retinocytoma is regarded as a rare variant of retinoblastoma with a more benign course due to an incomplete mutation of the retinoblastoma gene. The prognosis for retinocytoma is more favorable due to the presence of clear signs of differentiation in the form of the formation of true rosettes and a tendency to spontaneous regression.

To diagnose retinoblastoma, ophthalmoscopy is used, which should be performed when maximum pupil dilation, and in young children - during medicated sleep. When examining the fundus at the extreme periphery, it is necessary to apply sclerocompression, which allows for a more detailed examination of these areas that are difficult to access for visual inspection. Carry out ophthalmic

Pius follows all meridians(!). In difficult cases with a preequatorial tumor location or the presence of a pseudohypopyon, fine-needle aspiration biopsy is indicated. Ultrasound scanning allows you to determine the size of retinoblastoma and confirm or exclude the presence of calcifications.

Treatment of retinoblastoma is complex, aimed at preserving the life of the sick child and his eye, always individual, it is planned depending on the stage of the process, the general condition of the child, the risk of developing second malignant tumors and the ultimatum of the parents to preserve their vision. For small tumors, the use of local destruction methods makes it possible to save the eye, and in combination with polychemotherapy, to achieve a 5-year survival rate of almost 90%. For large tumors, polychemotherapy in combination with eye enucleation provides a 4-year survival rate of more than 90%. Retinoblastoma disseminates along the optic nerve through the interthecal space, spreads hematogenously to the bones and brain, and lymphogenously to regional lymph nodes.

In order to detect early tumor relapses after enucleation of the eyeball or the appearance of a tumor in the fellow eye, a follow-up examination of the child is required. For monolateral retinoblastoma, it should be performed every 3 months for 2 years, for bilateral retinoblastoma - for 3 years. In children older than 12 months, after completion of treatment, it is advisable to perform a computed tomography scan of the head once a year, which will allow monitoring the condition of the soft tissues of the orbits and excluding tumor metastasis to the brain. Cured children should be under medical supervision for life.

20.3.1.5. Orbital tumors

Almost all tumors observed in humans develop in the orbit.

Benign tumors. For

Benign tumors of the orbit are characterized by a general clinical picture: swelling of the eyelids, stationary exophthalmos, difficulty in repositioning and limitation of eye mobility, changes in the fundus, decreased vision, pain in the affected orbit and the corresponding half of the head. A small tumor located at the apex of the orbit may be asymptomatic for a long time.

Vascular tumors most often represented cavernous hemangioma. The tumor is detected at the age of 12-65 years, in women 2.5 times more often; it has a well-defined pseudocapsule. Microscopically, cavernous hemangioma consists of large dilated vascular canals lined with flattened endothelial cells; there is no direct transition of the tumor vessels into the vessels of the surrounding orbital tissues. Clinically, the tumor is characterized by slowly increasing stationary exophthalmos (Fig. 20.20, a). Its close location to the sclera leads to the formation of folded vitreous plate (Bruch's membrane) and dry dystrophic lesions in the paramacular region in the fundus (Fig. 20.20, b). Repositioning the eye, despite the sufficient elasticity of the hemangioma, is usually difficult. Deeper localization of cavernous hemangioma (at the apex of the orbit) may be accompanied by pain in the affected orbit and the corresponding half of the head. Usually, with such localization, a picture of a congestive optic disc or its primary atrophy appears early. The diagnosis is clarified using computed tomography, when

Rice. 20.20. Cavernous hemangioma of the right orbit.

a - the palpebral fissure is widened, exophthalmos with a slight displacement of the eyeball; b - fundus: folding of Bruch’s membrane (symptom of “transverse retinal striation”).

This reveals a cellular rounded shadow with smooth contours, delimited from the surrounding tissues by the shadow of the capsule. The intensity of the tumor shadow increases if it is contrasted. Ultrasound scanning reveals a tumor shadow limited by the capsule. Treatment is surgical. Taking into account the emerging possibilities of clear tumor topometry using computed tomography and the use of microsurgical techniques, it should be recommended to perform surgery immediately after diagnosis. There is no reason to expect clinical progression

ical symptoms, since their occurrence can lead to irreparable loss of visual functions.

Optic nerve tumors represented by meningioma and glioma.

Meningioma develops from arachnoid villi located between the dura mater and the arachnoid membrane. The diameter of the optic nerve affected by the tumor increases 4-6 times and can reach 50 mm (Fig. 20.21). Meningioma can grow into the optic nerve sheath and spread to the soft tissue of the orbit. As a rule, the tumor is one-sided, characterized by exophthalmos with early vision loss. When the orbital tissue grows, restriction of eye movements occurs. In the fundus there is a pronounced congestive optic disc, less often - its atrophy. The appearance of significantly dilated, bluish-colored veins on the stagnant disc indicates the spread of the tumor directly to the posterior pole of the eye.

Diagnosis of meningioma is difficult, since even on computer tomograms, especially in the initial stage of the disease, the optic nerve is not always enlarged enough to suggest tumor damage. Treatment is surgery or radiation therapy. The prognosis for vision is unfavorable. When a tumor grows along the optic nerve trunk, there is a threat of its spread into the cranial cavity and damage to the chiasma. The prognosis for life is favorable if the tumor is located within the orbital cavity.

Glioma consists of three types of cells: astrocytes, oligodendrocytes and macroglia. It is characterized by an extremely slow but steadily progressive decrease in visual acuity. In children under 5 years of age, the first sign that parents pay attention to is strabismus; nystagmus is possible. Exophthalmos

Rice. 20.21. Computer tomogram of the eye. Optic nerve meningioma. The shadow of an unevenly thickened optic nerve is visible; the tumor does not extend beyond the orbit.

national, painless, appears later and grows very slowly. It can be axial or displaced due to eccentric tumor growth. Repositioning the eye is always difficult. In the fundus, congestive disc or optic nerve atrophy is equally often detected. Intracranial spread is accompanied by dysfunction of the hypothalamus, pituitary gland, and increased intracranial pressure.

The diagnosis of glioma is made based on the results of computed tomography, which can not only visualize the enlarged optic nerve in the orbit, but also judge the spread of the tumor along the optic nerve canal into the cranial cavity (Fig. 20.22). Ultrasound scanning is not informative enough, since it represents only the proximal and middle thirds of the optic nerve. Given the extremely slow growth of the tumor, treatment is strictly individual. While maintaining vision and the possibility of monitoring the patient, an alternative to long-term

Rice. 20.22. Computer tomogram of the eye. Optic nerve glioma. The shadow of the fusiform thickened optic nerve is projected in the lumen of the orbit; the optic nerve canal is free of tumor.

Observation may include radiation therapy, after which stabilization of tumor growth or even improvement in vision is noted. Surgical treatment is indicated when the tumor affects only the orbital segment of the optic nerve. If the tumor grows to the scleral ring, which is clearly visible on CT scans, the affected optic nerve must be removed along with the eye. If the tumor spreads into the optic nerve canal or the cranial cavity, the question of the possibility of performing surgical intervention is decided by a neurosurgeon. The prognosis for vision is always poor, and for life it depends on the spread of the tumor into the cranial cavity. When the chiasm is involved in the tumor process, the mortality rate reaches 20-55%.

Neuroma(synonyms: lemmoma, schwannoma, neurolemmoma). In the orbit, the source of this tumor in most cases is the ciliary nerves, supratrochlear or supraorbital; it can also be formed from sympathetic lemmocytes.

tic nerves innervating the vessels of the meningeal membrane of the optic nerve and the retinal artery. One of the first signs of a tumor may be localized pain in the affected orbit, non-inflammatory swelling of the eyelids (usually the upper one), partial ptosis, and diplopia. In 25% of patients, the first thing that attracts attention is exophthalmos, axial or displaced. The tumor is most often located under the superior orbital wall in the external surgical space. The area of ​​cutaneous anesthesia suggests involvement of the supratrochlear or supraorbital nerve. In 1/4 of patients, corneal anesthesia is noted. A large tumor limits the mobility of the eye in the direction of its localization. In the fundus there is swelling of the optic nerve head. With ultrasound scanning, it is possible to visualize the shadow of the tumor, delimited by the capsule. A CT scan shows not only the shadow of the tumor, but also its relationship with the optic nerve. The final diagnosis is established after histological examination. Treatment is only surgical. An incompletely removed tumor is prone to recurrence. The prognosis for vision and life is favorable.

Congenital neoplasms. Neoplasms of this group include dermoid and epidermoid (cholesteatoma) cysts. Their growth is accelerated by trauma, and cases of malignancy have been described.

Dermoid cyst It grows extremely slowly, but during puberty and during pregnancy there are cases of its rapid increase. The cyst develops from wandering epithelial cells, concentrated near the bone sutures, and is located under the periosteum. The epithelium of the inner wall of the cyst secretes mucous contents

Mine is mixed with cholesterol crystals, which gives it a yellowish tint. The cyst may contain short hairs. Favorite localization is the area of ​​bone sutures. Patients' complaints, as a rule, boil down to the appearance of painless swelling of the upper eyelid, depending on the location of the cyst. The skin of the eyelid in this area is slightly stretched, but its color is not changed; an elastic, painless, immobile formation is palpated.

It is extremely rare that a dermoid cyst is localized deep in the orbit. This is the so-called pouch-shaped dermoid cyst of the orbit.

Surgical treatment: subperiosteal orbitotomy is indicated. The prognosis for life and vision is favorable. Relapse should be expected with pronounced changes in the underlying bones or the appearance of a postoperative fistula. Recurrent dermoid cysts can become malignant.

Cholesteatoma, or epidermoid cyst, develops in the fourth decade of life. The etiology and pathogenesis are unclear. Cholesteatoma is characterized by local fibering of bone tissue, which becomes soft; areas of osteolysis increase. As a result of detachment of the periosteum by the underlying necrotic masses, the volume of the orbit decreases. Painless exophthalmos gradually develops. Reposition is difficult. There are no changes in the fundus, the functions of the extraocular muscles are preserved in full. The diagnosis is made based on the results of radiography and computed tomography. Treatment is only surgical. The prognosis for life and vision is usually favorable, but cases of malignancy of the cyst have been described.

Pleomorphic adenoma (mixed tumor) - a benign variant of the primary tumor of the tears -

no gland. The tumor consists of two tissue components: epithelial and mesenchymal. The epithelial component forms areas of mucus- and chondro-like structures. The stroma (mesenchymal component) is heterogeneous within one node: sometimes it is loose, in some areas there are connective tissue strands, and areas of hyalinosis are noted. The tumor develops slowly. Patients consult a doctor 2-32 years after the onset of clinical symptoms. One of the first is painless, non-inflammatory swelling of the eyelids. Gradually, the eyeball moves downward and inward. Exophthalmos occurs much later and increases very slowly. During this period, it is already possible to palpate a fixed formation located under the upper outer or upper edge of the orbit. The surface of the tumor is smooth, upon palpation it is painless and dense. Reposition is difficult.

X-rays reveal an increase in the orbital cavity due to thinning and upward and outward displacement of its wall in the area of ​​the lacrimal fossa. Ultrasound scanning allows you to determine the shadow of a dense tumor surrounded by a capsule and the deformation of the eye. With computed tomography, the tumor is visualized more clearly; it is possible to trace the integrity of the capsule, the extent of the tumor in the orbit, and the condition of its adjacent bone walls. Treatment is only surgical. The prognosis for life and vision in most cases is favorable, but the patient should be warned about the possibility of relapse, which can occur after 3-45 years. The risk of malignant degeneration of pleomorphic adenoma increases as the period of remission increases.

Malignant tumors orbits, like benign ones, are poly-

are morphic in histogenesis, the degree of their malignancy is variable. Sarcomas and cancer are more common. General clinical symptoms of malignant tumors of the orbit are early-onset diplopia and swelling of the eyelids, initially transient, appearing in the morning, and then becoming stationary. These tumors are characterized by constant pain, exophthalmos occurs early and grows quite quickly (over several weeks or months). Due to compression of the neurovascular bundle by the growing tumor, deformation of the eye and large exophthalmos, the protective function of the eyelids is disrupted: in these patients, dystrophic changes in the cornea rapidly increase, ending in its complete melting.

Cancer in the orbit, in the vast majority of cases it develops in the lacrimal gland, less often it is represented by heterotopic cancer or embryonal infantile carcinoma.

Lacrimal gland cancer It has significant cellular variability and grows quickly. The history of the disease usually does not exceed 2 years; more often, patients note an accelerated increase in all symptoms over several (up to 6) months. Often the first symptoms of developing cancer are pain, discomfort, lacrimation, and uneven drooping of the upper eyelid. Exophthalmos develops with a displacement of the eyeball downward and inward. Astigmatism develops as a result of mechanical deformation of the eye by a tumor. The palpable tumor is lumpy and practically does not move in relation to the underlying tissues. Movement of the eye towards the location of the tumor is limited, reposition is severely difficult.

The diagnosis is made only after histological examination. Presumable diagnosis

can be established based on an analysis of clinical symptoms and the results of instrumental research. X-rays reveal areas of bone destruction against the background of an enlarged orbit. Computed tomography allows us to determine the extent of the tumor shadow, spread into the adjacent extraocular muscles and the unevenness of the contours of the bone wall of the orbit or its complete destruction, and ultrasound allows us to determine only the presence of the tumor shadow and its density. A radioscintigram of the orbit in adenocarcinoma is characterized by an increase in the asymmetry coefficient characteristic of malignant tumors. Remote thermography is informative, especially with a sugar load. Preoperative fine-needle aspiration biopsy helps clarify the diagnosis.

Treatment of lacrimal gland cancer is a complex task. Along with the radical point of view, whose supporters require mandatory exenteration of the orbit, there is an opinion about the possibility of organ-preserving treatment, combining excision of the tumor with a block of adjacent healthy tissue and postoperative external irradiation of the orbit. The choice of a particular treatment method depends on the size of the tumor and its prevalence. If the integrity of the orbital bones is compromised, surgical treatment, including exenteration, is contraindicated. The prognosis for life and vision is poor, since the tumor is not only prone to recurrence with invasion into the cranial cavity, but also to metastasis to the lungs, spine or regional lymph nodes. The timing of metastases varies from 1-2 to 20 years.

Primary orbital cancer is rare. The tumor grows slowly, without a capsule. When localizing the tumor

Holly in the anterior part of the orbit, the first symptom is a displacement of the eye in the direction opposite to the localization of the tumor, its mobility is sharply limited, exophthalmos slowly increases, which never reaches large degrees, but reposition of the eye turns out to be impossible. Congestion occurs in the episcleral veins, and ophthalmotonus increases. The eye seems to be walled up by the tumor in the orbit, its bony edges are “smoothed out” by the surrounding tumor and become inaccessible to palpation. Visual functions are preserved for a long period of time, despite secondary intraocular hypertension.

When the tumor is localized at the apex of the orbit, pain occurs early and radiates to the corresponding half of the head. As the tumor grows, complete ophthalmoplegia occurs. Characterized by primary atrophy of the optic nerve head with a rapid decrease in visual function. Exophthalmos is noticed late and usually does not reach high levels.

Features of the clinical picture and the results of ultrasound scanning make it possible to determine the infiltrative spread of the pathological process without revealing its nature. Computed tomography demonstrates a dense, infiltratively growing tumor and its relationship with the surrounding soft tissue and bone structures of the orbit. The results of radionuclide scintigraphy and thermography indicate the malignant nature of tumor growth. Fine-needle aspiration biopsy with cytological examination of the obtained material is possible if the tumor is localized in the anterior orbit. If it is located at the apex of the orbit, aspiration is associated with the risk of eye damage.

Surgical treatment (subperiosteal exenteration of the orbit) is indicated for anterior localization of the process. Treatment of more deeply located tumors is difficult, since they grow into the cranial cavity early. Radiation therapy is ineffective because primary heterotopic cancer is refractory to radiation. The prognosis is bad.

Sarcomas - the most common cause of primary malignant growth in the orbit.

Rhabdomyosarcoma- An extremely aggressive tumor of the orbit, it is the most common cause of malignant growth in the orbit in children.

The favorite location of the tumor is the upper inner quadrant of the orbit, so the levator palpebral muscle and the superior rectus muscle are early involved in the process. Ptosis, restriction of eye movements, downward and inward displacement of the eye are the first signs that are noticed by both the patients themselves and those around them. In children, exophthalmos or displacement of the eye when the tumor is localized in the anterior orbit develops within several weeks (Fig. 20.23, a). In adults, the tumor grows more slowly, over several months. A rapid increase in exophthalmos is accompanied by the appearance of congestive changes in the episcleral veins, the palpebral fissure does not close completely, infiltrates on the cornea and its ulceration are noted. In the fundus there is a congestive optic disc. The tumor quickly destroys the adjacent thin bone wall and grows into the nasal cavity, causing nosebleeds. Ultrasound scanning, computed tomography, thermography and fine-needle aspiration biopsy are the optimal diagnostic complex of instrumental methods.

Rice. 20.23. Rhabdomyosarcoma of the right orbit.

a - non-inflammatory swelling of the eyelids, exophthalmos with displacement of the eyeball downward and inward; b - computed tomogram: shadow of a tumor with unclear and uneven boundaries in the area of ​​the external rectus muscle.

dov studies in rhabdomyosarcoma (Fig. 20.23, b). Combined treatment. The treatment protocol provides for preliminary polychemotherapy for 2 weeks, after which external irradiation of the orbit is performed. After combined treatment, 70% of patients live for more than 3 years.

Malignant lymphoma is currently considered a neoplasm of the immune system. The process involves predominantly lymphoid elements of T- and B-cell affiliation, as well as elements of zero populations. Morphological examination in the orbit more often diagnoses

Rice. 20.24. Malignant lymphoma of the right orbit.

a - ptosis of the upper eyelid, the palpebral fissure is narrowed, exophthalmos with displacement; b - fundus: congestive optic disc; c - computed tomogram of the eye: the shadow of a tumor filling almost the entire orbital cavity.

They are low-grade B-cell lymphoma, which contains fairly mature tumor and plasma cells. Typically, one orbit is affected. Characterized by the sudden appearance of painless exophthalmos and swelling of the periorbital tissues. Exophthalmos can be combined with ptosis (Fig. 20.24, a). The process locally progresses steadily, red chemosis develops, eye reposition becomes impossible, changes occur in the fundus, most often a congestive optic disc (Fig. 20.24, b). Vision decreases sharply. During this period, pain may appear in the affected orbit.

The diagnosis of malignant orbital lymphoma is difficult. Of the instrumental research methods, ultrasound scanning, computed tomography (Fig. 20.24, c) and fine-needle aspiration biopsy with cytological examination are more informative. An examination by a hematologist is necessary to exclude systemic damage. External irradiation of the orbit is practically no alternative, highly effective treatment method. Polychemotherapy is used for systemic lesions. The effect of treatment is manifested in the regression of symptoms of the pathological process in the orbit and the restoration of lost vision. The prognosis for primary malignant lymphoma for life and vision is favorable (83% of patients survive the 5-year period). The prognosis for life worsens sharply with disseminated forms, but they are rare.

Questions for self-control

1.Name the signs of progressive nevus of the skin of the eyelids.

2. List the clinical signs of basal cell skin cancer of the eyelids.

4.Name the signs of eyelid skin melanoma. Based on what symptoms can you distinguish it from a nevus? Is surgical treatment possible for melanoma of the eyelid skin?

5. List the signs of progressive conjunctival nevus.

6.Name the signs of progressive growth of iris leiomyoma.

7. List the signs that allow you to differentiate between nevus and pigmented leiomyoma of the iris.

8.What are the causes of cataracts in melanoma of the iridociliary region and ciliary body?

9. Name the signs of progression of choroidal nevus. What is the doctor’s tactics in this case?

10. List the sources of development of choroidal melanoma.

11.Why do visual disturbances develop in choroidal melanoma?

12.Is enucleation indicated in all cases of choroidal melanoma?

13.What are the causes of familial retinoblastoma?

14.What can explain the high risk of developing second malignant tumors in patients with retinoblastoma?

15. Is it possible for indirect signs to appear in early retinoblastoma?

16. Is eye enucleation the only treatment for retinoblastoma?

3089 0

Tumors of the choroid make up the majority of intraocular neoplasms and occur between the ages of 3 and 80 years.

About 23% of all tumors are localized in the iris and ciliary body, the rest are in the choroid.

Their histogenesis is varied, but the main part is of neuroectodermal origin (pigmented and non-pigmented tumors), a smaller part of them belongs to tumors of mesodermal origin (vascular neoplasms).

Up to 84% of iris tumors are benign, most of them are of myogenic nature, which was proven in 1973 by E.F. Levkoeva. Somewhat earlier, Heals (1952) and Ashton (1963) showed a lower frequency of myogenic tumors in the iris (2.3-14.5%). G.G. Ziangirova (1980), using large material and long-term observation of patients, convincingly proved the predominance of fibroids (54%) among all iris tumors.

We found confirmation of her data in our long-term observations of 476 patients with tumors of the iris and ciliary body, among which benign tumors accounted for 83.23%, and myogenic among them - 62%. Malignant tumors are represented overwhelmingly by melanoma and metastatic tumors.

Leiomyoma

The tumor develops from elements of the pupillary muscles and is characterized by extremely slow growth. Depending on the presence of pigment in the tumor, non-pigmented and pigmented leiomyomas are distinguished.

This division is explained by different clinical pictures. The tumor is diagnosed almost equally often in men and women, mainly in the 3rd-4th decade of life. In recent years, cases of detection of leiomyomas in girls aged 16-20 have become more frequent.

Clinic

Amethymic leiomyoma

It grows locally in the form of a yellowish-pink, translucent, proliferating node. The tumor can be localized along the pupillary edge, in the zone of Krause's steep slope, in the hilar zone (in the area of ​​ciliary crypts). The boundaries of leiomyoma are quite clear, the consistency is loose, gelatinous (Fig. 6.1).

Rice. 6.1. Pigmentless iris leiomyoma

On its surface, translucent outgrowths are visible, in the center of which there are vascular loops. These newly formed vessels can be a source of recurrent hemorrhages into the anterior chamber during tumor growth.

With non-pigmented leiomyoma in the area of ​​ciliary crypts, one of the first symptoms that forces the patient to see a doctor is an increase in intraocular pressure (IOP) with all its typical signs. Often such patients are diagnosed with unilateral primary glaucoma.

Pigmented leiomyoma

It has a color from light brown to dark brown, and can be nodular, planar or mixed in growth form (Fig. 6.2).

Rice. 6.2. Leiomyoma of the iris. a - appearance of pigmented leiomyoma; b - histological specimen

Most often localized in the ciliary belt of the iris. Characteristic changes in the shape of the pupil, its lengthening due to eversion of the pigment border directed towards the location of the tumor. The consistency of pigmented leiomyoma is denser, the surface is lumpy, and newly formed vessels are not visible.

Tumor growth into the angle of the anterior chamber by more than 1/3 of its circumference leads to the appearance of secondary intraocular hypertension. Given the extremely slow growth of leiomyomas, it is important to know the signs of tumor progression.

These include:

1) smoothness of the iris relief around the tumor due to perifocal atrophy of its tissue;
2) the appearance of a zone of sputtering of pinpoint pigment around the tumor and the formation of pigment tracks away from the peripheral boundaries of the tumor;
3) a change in the shape of the pupil with a violation of its reaction in this zone to light and milriatics;
4) the appearance of a vascular corolla in the iris around the tumor.

Leiomyomas of the iris, as a rule, are limited in their growth to the anterior chamber and grow into the posterior chamber extremely rarely. When spreading into the structures of the angle of the anterior chamber and the ciliary body, the tumor exits into the posterior chamber with displacement and clouding of the lens.

Morphogenesis

For many years, the literature has been discussing the histogenesis of leiomyomas. Judgments are made about the rarity of this tumor, and a high frequency of melanomas in the iris is indicated. Meanwhile, G.G. Ziakgirova wrote in 1980 that it is possible to understand the morphological features of muscle tumors only by comparing them with the morphogenesis of the iris: the pupillary muscles of the iris are formed from the outer layer of the pigment epithelium of the iridociliary rudiment.

In embryogenesis, pupillary sphincter cells produce melanin for myofibrils; in postnatal life, this ability disappears. The pupillary dilator retains its ability to synthesize melanin in the postnatal period.

This can explain the absence of pigment in leiomyomas developing from the sphincter of the pupil and its presence in leiomyomas formed from elements of the pupillary dilator. Histologically, leiomyomas are characterized by the presence of long, spindle-shaped, well-differentiated cells. The nuclei are oval or rod-shaped, without inclusions and nucleoli. The presence of myofibrils is characteristic.

In non-pigmented forms, thin-walled, newly formed, telangiectatic dilated vessels are visible. In pigmented leiomyomas, neuroepithelial cells are found, the more of them the more pronounced the pigmentation. Large conglomerates of pigment granules are present in the cytoplasm.

The diagnosis of leiomyoma is established on the basis of biomicroscopy and gonioscopy data. Diaphanoscopy is also informative in non-pigmented forms, when a defect in the iris is clearly visible against the background of a yellowish tumor node. Fluorescein angiography (FAH) has characteristics characteristic of leiomyoma. Immunohistochemical studies reliably document the diagnosis of leiomyoma

Differential diagnosis

Pigmentless leiomyoma requires differentiation from mesodermal dystrophy of the iris, chronic nonspecific granuloma, sarcondosis, juvenile xanthogranuloma, adenocarcinoma, metastasis, and abscess. Pigmented leiomyoma must be differentiated from a progressive nevus, melanoma, pigment leaf cyst, or encapsulated foreign body.

Treatment

Considering the possibility of progression of tumor growth with the development of secondary glaucoma, cataracts, leiomyomas are subject to surgical treatment - local removal with a surrounding block of healthy tissue. If no more than 1/3 of the iris circumference is removed, its integrity can be restored by applying microsutures (Fig. 6.3).

Rice. 6.3. Leiomyoma of the iris. a - appearance of the eye before treatment; b - the same eye after removal of the tumor with a one-stage iris plate

Many years of experience testify to this. that restoring the integrity of the iris, like a diaphragm, sharply reduces the degree and frequency of lens astigmatism and reduces light aberrations (Fig. 6.4).

Rice. 6.4. Functional outcomes after local resection of iridocylcarcinoma tumors

The prognosis for life is favorable. The prognosis for vision depends on the initial size of the tumor. The smaller the tumor, the more likely the patient can be promised the preservation of normal vision.

Epithelioma

Epithelioma, or pigment epithelial adenoma, is extremely rare. It is diagnosed equally often in men and women, usually in the 2nd-3rd decade of life, which can be explained by the extremely slow growth of the tumor.

Clinic

The tumor is localized mainly in the ciliary belt of the iris. Epithelioma, as a rule, has an intensely dark, almost black color (Fig. 6.5. a, b).

Rice. 6.5. Epithelioma of the iris. a - appearance of the tumor. b - spread of the tumor into the structure of the eye. c - histological specimen

As the tumor grows, it displaces the atrophic stroma of the iris anteriorly and grows into it. As a rule, epithelioma is represented by a single node with raised edges; sometimes the edges of the tumor gradually merge into healthy iris tissue, which makes it look like melanoma.

Small pigment cysts may be observed along the edges of the tumor. Epithelioma grows slowly and can grow into the posterior chamber, displacing and squeezing the lens, which leads to the formation of cataracts. Epithelioma often grows into the structures of the anterior chamber angle, which is complicated by secondary glaucoma.

Morphogenesis

Epithelioma develops from pigment epithelial cells and consists of mature, uniform large polygonal cells with massive cytoplasm filled with melanin granules and vacuoles. There are no cell mitoses, but the tumor is characterized by invasive growth (Fig. 6.5, c).

The diagnosis of epithelioma is established on the basis of biomicroscopy and gonioscopy data. FA and radiophosphorus test are not informative. The final diagnosis is confirmed by histological examination.

Differential diagnosis

It is necessary to carry out with pigmented leiomyoma, melanoma, metastasis, nevus, melanocytoma, pigment cyst, foreign body, granuloma.

Treatment

Despite their slow growth, iris epitheliomas are subject to local removal. Taking into account the benign nature of the growth, local excision with replacement of the tissue defect is possible if the tumor has spread to the episclera.

The prognosis for life is good. The tumor does not metastasize. Preservation of vision depends on the size of the tumor and existing complications.

Medulloepithelioma (dictyoma)

A tumor in the iris is found in children aged 6 months to 6 years and is classified as a congenital neoplasm. In recent years, it has been regarded as a developmental defect.

Clinic

On the surface of the iris, the tumor has the appearance of translucent whitish-gray flakes, loosely interconnected, which creates conditions for their movement along the anterior chamber. In this case, the outflow paths of chamber humor may become obstructed or the pupil area may close with the development of secondary glaucoma.

A.Moonis and A.Gamer (1975) described a case of medulloepithelioma of the iris in a 3-year-old child, which looked like an isolated intensely pigmented tumor with a tuberous surface, filling the lower half of the anterior chamber and fused with posterior corneal epithelium(ZER). The tumor can grow along the posterior surface of the iris, mainly in its lower half, and enter the anterior chamber through the pupil.

Morphogenesis

Medulloepithelioma originates from primitive germinal epithelium.

The diagnosis is made on the basis of biomicroscopy. In particularly difficult cases it is possible fine needle aspiration biopsy (TIAB).

Differential diagnosis should be made with retinoblastoma, non-pigmented melanoma, metastasis, inflammatory granuloma, aseptic abscess of the anterior chamber.

Treatment

For a localized node, block excision is indicated. Tumors that occupy more than half the circumference of the iris and extend to the ciliary body, retina, or beyond the fibrous capsule can be cured only by enucleation.

The prognosis for life is good even if atypical cells and mitoses are detected in histological specimens. The tumor does not metastasize. The preservation of vision depends on the size of the tumor and the involvement of neighboring tissue structures in the process.

Hemangioma

Hemangioma is a rare congenital tumor. Clinically manifests itself both in the first years of life and after the 2-3rd decade. Iris hemangiomas have been described in patients in the 6th-7th decade of life. In our 6 observations, hemangioma detected itself after 20 years, more often in men.

There is reason to believe that oka is combined with vascular systemic damage to organs and tissues. Like vascular tumors of other localizations, capillary, cavernous and racemotic hemangiomas are observed in the iris.

Clinic

Capillary hemangioma is a tumor of newborns or children in the first years of life. Presented in the form of intertwining vessels growing into the iris. The tumor is reddish-bluish in color and occupies from one quadrant of the iris to half of its surface. Such hemangiomas can regress on their own. With continued growth, secondary glaucoma develops.

Cavernous hemangioma is most often located at the pupillary edge and is represented by a brownish-yellow or reddish node. Depending on the filling of the tumor cavities with blood, its prominence changes. Transient hyphemas are characteristic. With hyphema of more than 1/2 of the volume of the anterior chamber, IOP increases and corneal edema appears.

Racemotic hemangioma in the iris is extremely rare and is manifested by sharply tortuous, dilated, intertwining vessels located in the thickness of the iris and extending into the corner of the anterior chamber. Secondary intraocular hypertension develops early.

Morphogenesis

Being a developmental defect, hemangioma is histologically represented by newly formed thin-walled vessels at different stages of development.

The diagnosis is made on the basis of biomicroscopy.

Differential diagnosis includes iris neovascularization, melanoma, juvenile xanthogranuloma, sarcondosis, amethyst leiomyoma, and metastasis.

Treatment

Local treatment is effective for cavernous hemangioma in the form of laser coagulation of the tumor. Capillary hemangiomas can regress spontaneously. Racemotic hemangiomas are practically untreatable.

The prognosis for life is good. It is beneficial for vision in cases of cavernous hemangioma. With capillary and racemotic hemangiomas, the prognosis for vision is uncertain.

Nevus

The color of the iris and the pattern of the crypts are genetically programmed for each person. The nature of its color in almost everyone is heterogeneous: areas of hyperpigmentation in the form of small spots or, as they are called, “freckles” are scattered across the surface of the iris.

True nevi are classified as melanocytic tumors, which occur in both children and adults. They are usually discovered by accident. When talking with patients, it is possible to find out that the speck on the iris was noticed in childhood. Among the 476 patients with tumors of the iris and ciliary body observed in our clinic, nevi accounted for 2.5%.

Clinic

A nevus is represented by an area of ​​hyperpigmentation of the iris, the color of which varies from yellow to intense brown. The surface of the tumor is velvety, slightly rough. Sometimes the nevus protrudes slightly above the surface of the iris. Its boundaries are clear, the pattern of the iris on the surface of the nevus is smoothed (Fig. 6.6).

Rice. 6.6. Iris nevus

In the central part the tumor is denser and the iris pattern is absent. The size of the nevus ranges from 2-3 mm to large lesions occupying up to one quadrant of the iris. Stationary nevi do not change their size, shape and color for decades.

When signs of progression appear, the tumor darkens, increases in size, a spray of previously unobserved pigment appears around the nevus, the boundaries of the formation become less clear, and a rim of dilated vessels appears around the tumor.

Morphogenesis

Iris nevi are derivatives of the neural crest and are morphologically represented by an accumulation of mature melanocytes, spindle-shaped cells similar to Schwann elements, and large dense polygonal cells. There are many melanin granules in the cytoplasm of cells. Tumor growth is diffuse but limited to the superficial layers of the iris or the outer layers of the stroma.

Treatment of stationary nevi is not required. If the nevus progresses, local excision is indicated.

The prognosis for life and vision is good.

Melanoma

Melanocytoma - a large cell (or magnocellular) nevus in the iris is very rare. Characterized by an intense black color. The tumor grows slowly, but over the course of several months it can sharply increase in size due to spontaneous necrosis with dissemination of pigment and perifocal inflammation in the surrounding tissues. Clinically, this is manifested by a sharp increase in intraocular pressure.

Histology

The nevus is represented by round, large, mature melanocytes.

Diagnosis

Biomicroscopy helps in diagnosis. In difficult cases, TIAB can be used.

Differential diagnosis includes melanoma, epithelioma and pigmented leiomyoma.

Treatment

As a rule, patients do not need treatment. If IOP increases, local excision of the tumor is possible.

The prognosis for life and vision is favorable.

Iris nevus syndrome

Kogan-Rhys syndrome - iris-nevus syndrome - occurs more often in women and is characterized by the appearance in the iris of multiple nevus-like nodules on a stalk against the background of abnormal pigmentation (heterochromia).

Typical is the development of peripheral anterior synechiae, proliferation of the corneal endothelium over the surface of the iris, and the appearance of unilateral glaucoma with decreased vision. Unlike mesodermal dystrophy of the iris, there is no stromal rarefaction in iris nevus syndrome. M.Khalil and M.Finlayson (1980) morphologically found ectopic corneal endothelium covering the anterior surface of the iris in such a patient.

In the latter, an accumulation of melanophagic cells was found. According to T. Makley (1988), the only treatment method for iris nevus syndrome should be considered antiglaucomatous surgery, which, unfortunately, leads to temporary success.

A.F. Brovkina, V.V. Valsky, G.A. Gusev

Leiomyoma is a benign formation of mutated smooth muscle fibers. In most cases, such a tumor does not become malignant, however, if there is a predisposition to cancer, transformation into a malignant form should not be excluded.

Leiomyoma has virtually no restrictions regarding location - the tumor can form in the uterus (the most common option for women), in the intestines, in the esophagus, in the lungs, and the membranes of the eye are not excluded. Despite the fact that the formation is characterized by a benign course, excision is mandatory. However, even in this case, relapse cannot be ruled out.

The clinical picture of the pathological process depends on where exactly the tumor forms in the body. Therefore, the symptoms in this case are nonspecific. Treatment is individualized, but in most cases surgery is performed to remove the tumor.

The prognosis is often relatively favorable. Much depends on which part of the tumor formed and how quickly treatment was started. General health indicators and the patient’s age are also taken into account.

Etiology

The exact reasons for the development of such a pathological process have not yet been established.

However, clinicians identify the following predisposing factors:

• hereditary predisposition;
• previous oncological diseases;
• the presence of systemic diseases, chronic with frequent relapses;
• thyroid diseases and other pathological processes that lead to hormonal imbalances;
• ophthalmological diseases, eye injuries;
• decreased immunity due to prolonged infectious pathologies or;
• gastroenterological diseases of a chronic nature with frequent relapses;
• previous abdominal surgeries;
• a sedentary lifestyle combined with frequent exercise;
• uncontrolled use of hormonal drugs, antibiotics, corticosteroids;
• disturbances in the functioning of the brain, namely in the areas that are responsible for the production of hormones;
• chronic urological diseases, late onset of sexual activity.

Due to the fact that specific etiological factors have not been established, unfortunately, there is no specific prevention.

Classification

This pathological process is classified according to two criteria: the number of tumor nodes and their location in the body.

Based on quantitative characteristics, the following forms are distinguished:

• solitary or solitary leiomyoma;
• multiple.

Based on the localization of the tumor formation, the following forms are considered:

• Submucosal or submucosal leiomyoma (submucosal leiomyoma) is diagnosed quite rarely and cannot be asymptomatic, especially if it is located in the uterus or small intestine area. The tumor is similar to a polyp - a body with a stalk that is attached to the submucosal layer.
• Intermuscular or intramural leiomyoma is one of the most common forms of the pathological process. In addition to general symptoms, it leads to circulatory problems.
• Subperitoneal or subserous leiomyoma (leiomyoma of the retroperitoneal space) - in some cases it can regress without specific treatment.
• Intraligamentary leiomyoma - tumor nodes, as a rule, form between the uterine ligaments. This form of the disease is rare.
• Cervical nodular leiomyoma is a rare form of the pathological process. If a tumor of this type forms in the uterus, it can lead to infertility.

A classification is also used, from the point of view of the histological structure of this formation:

• cellular leiomyoma;
• miotic;
• hemorrhagic – most often manifests itself after taking hormonal drugs, characterized by swelling and hemorrhage;
• leiomyolipoma - formation is characterized by a high fat content;
• myxoid – the tumor contains mucus, which can degenerate into a malignant neoplasm;
• vascular – there are vessels inside the tumor, which makes its removal more difficult;
• epithelioid (“bizarre”) - consists of round, epithelial-like cells.

The most common leiomyomas are the uterus, skin, gastrointestinal tract, and lungs. However, the development of pathology in other organs (for example, renal leiomyoma) is not excluded, although it is rare.

The nature of the course of the disease and the histological structure of the tumor can only be determined through diagnostics. It is impossible to assume this based on clinical signs alone.

Symptoms

At the initial stage of formation formation, there are, as a rule, no symptoms. As the tumor grows, the clinical picture will also appear. The symptom complex will depend on exactly where the pathological process develops.

Gastric leiomyoma will be characterized by the following clinical picture:

• “Hungry” pain – pain in the stomach occurs when a person has not eaten anything for 2-3 hours. After eating, the pain goes away.
• Nausea and vomiting. The vomit has the consistency of coffee grounds.
• Acute pain is localized not only in the abdomen, but also in the pelvic area. The manifestation of such a symptom will indicate tumor growth.
• Impaired stool frequency and consistency. The stool turns black due to internal bleeding.
• Paleness of the skin.
• Loss of body weight.
• Decreased appetite.
• If there are chronic gastroenterological diseases, then a relapse is possible due to a decrease in the body’s protective functions.

Due to the fact that esophageal leiomyoma is localized, as a rule, the transition to other organs does not occur. Therefore, symptoms relate only to the area in which tumor formation occurs.

Rectal leiomyoma has a similar clinical picture, but in addition there will be symptoms such as:

• pain in the anus during bowel movements;
• possible bloody discharge from the anus;
• feeling that there is a foreign body in the rectum.

Only a doctor can determine exactly what exactly provoked the appearance of such a clinical picture - esophageal leiomyoma or some other intestinal disease - by carrying out diagnostic measures. Therefore, if you have such a clinical picture, you need to contact a gastroenterologist.

Skin leiomyoma is also quite common, so it would be appropriate to give its clinical picture:

• the formation is located in the thickness of the dermis, size no more than 1.5 cm;
• appears above the skin in the form of a pink tubercle, sometimes with a brown tint;
• the tumor border is clear, the consistency is dense;
• painful palpation;
• in the cold the pain intensifies;
• Intense pain may occur, which goes away on its own after 1.5-2 hours.

It should be noted that a tumor of this type can be located not only on visible parts of the body, but also on the skin in the genital area.

Leiomyoma of the lungs is characterized by the following symptomatic complex:

• cough;
• temperature increase;
• general deterioration of health;
• shallow, wheezing breathing;
• frequent

Leiomyoma of the iris may be accompanied by the following clinical picture:

• local change in the color of the iris that was not there before;
• hemorrhages in the eyeball may periodically occur;
• increased intraocular pressure;
• clouding of the lens, which can lead to the development of ;
• blurred vision, increased lacrimation.

If treatment is not started in a timely manner, it will ultimately lead to complete destruction of the eye as an organ of vision. Blindness in this case is irreversible.

In general, the symptoms of such a pathological process are often nonspecific, so if you have any symptoms, you should consult a doctor who will prescribe an examination and effective treatment.

Diagnostics

First of all, a physical examination of the patient is carried out, collecting a personal and family history and studying the full clinical picture.

The following laboratory and instrumental tests are also prescribed:

• general clinical and biochemical blood test - hemoglobin and hematocrit are taken into account;
• tumor marker test;
• CT, MRI;
• biopsy of the tumor for further histological examination (a microslide is used).

Based on the results of diagnostic measures, treatment tactics will be determined.

Treatment

When treating this type of pathology, only an integrated approach is used - surgical removal of the tumor is combined with medication. Chemotherapy only takes place if the tumor becomes malignant.

Drug treatment can be prescribed both before and after surgery.

The following pharmacological groups are used:

• calcium channel blockers;
• alpha-adrenergic receptor inhibitors;
• vitamin and mineral complexes to strengthen the immune system.

If gastric leiomyoma is diagnosed, an additional diet is prescribed. The specific diet plan is determined by the doctor based on the current clinical picture.

The prognosis in most cases is relatively favorable if treatment is started in a timely manner. The exception is the myxoid form of the tumor, which tends to degenerate into malignant.

There are no specific methods of prevention. The only advisable solution would be a preventive medical examination every six months for early diagnosis of the disease.