Malignant tumors of the eye socket

Tumors of the orbit make up 23-25% of all neoplasms of the visual organ. Almost all tumors observed in humans develop in it. The frequency of primary tumors is 94.5%, secondary and metastatic - 5.5%.

Primary malignant tumors of the orbit make up no more than 0.1% of all malignant tumors in humans, while in the group of all primary tumors - 20-28%. They develop with almost equal frequency in all age groups. Men and women get sick equally often. Malignant tumors of the orbit, like benign ones, are polymorphic in histogenesis, the degree of their malignancy is variable. Sarcomas and cancer are more common. Common clinical symptoms of malignant tumors of the orbit are early-onset diplopia and eyelid edema, initially transient, appearing in the morning, and then becoming stationary. These tumors are characterized by constant pain syndrome, exophthalmos occurs early and increases quite quickly (within several weeks or months). Due to compression of the vascular-nerve 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.

Orbital cancer in the vast majority of cases develops in the lacrimal gland; less commonly, it is represented by heterotopic cancer or embryonic infantile carcinoma.

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Primary orbital cancer

Primary orbital cancer is rare. Men are more often affected. Primary orbital cancer is usually considered to be the result of tumor transformation of dystopic epithelial cells into soft tissues of the orbit. The tumor grows slowly, without a capsule. The clinical picture depends on its initial localization. When the tumor is localized in the anterior part of the orbit, the first symptom is the displacement of the eye to the side opposite to the tumor localization, its mobility is sharply limited, exophthalmos slowly increases, which never reaches large degrees, but reposition of the eye is impossible. A dense infiltratively growing tumor compresses the venous pathways of the orbit, resulting in congestion in the episcleral veins, and ophthalmotonus increases. The eye is walled up by the tumor in the orbit, its bone edges are "smoothed" by the surrounding tumor and become inaccessible to palpation. Visual functions are preserved for a long period of time, despite secondary intraocular hypertension.

Initial growth of the tumor at the orbital apex is manifested by early pain in it with irradiation to the corresponding half of the head and diplopia. As the tumor grows, complete ophthalmoplegia occurs. Primary atrophy of the optic nerve disc with a rapid decrease in visual functions is characteristic. Exophthalmos is noticed late, usually it does not reach high degrees.

The clinical features and ultrasound scan results allow us to determine the infiltrative spread of the pathological process without disclosing its nature. Computer tomography demonstrates a dense, infiltratively growing tumor, its relationship with the surrounding soft tissue and bone structures of the orbit. The results of radionuclide scintigraphy and thermography indicate a 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 part of the 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 deeper tumors is difficult, as they grow into the cranial cavity early. Radiation therapy is ineffective, as primary heterotopic cancer is refractory to radiation. The prognosis is poor. With anterior localization, the tumor metastasizes to regional lymph nodes. Cases of hematogenous metastasis are unknown, but with early tumor growth into the underlying bones and cranial cavity, the process becomes almost uncontrollable.

Sarcomas are the most common cause of primary malignant growth in the orbit. They account for 11-26% of all malignant orbital tumors. Almost any tissue can be the source of sarcoma development in the orbit, but the incidence of its individual types varies. The age of patients ranges from 3-4 weeks to 75 years.

Orbital rhabdomyosarcoma

Orbital rhabdomyosarcoma is an extremely aggressive orbital tumor and is the most common cause of malignant growth in the orbit in children. Boys are affected almost twice as often. The source of rhabdomyosarcoma growth is skeletal muscle cells. Three types of tumor are distinguished: embryonic, alveolar and pleomorphic, or differentiated. The latter type is rare. In children under 5 years of age, the embryonic type of tumor develops more often, after 5 years - alveolar. As a rule, rhabdomyosarcoma consists of elements of several types (mixed variant). An accurate diagnosis can only be established based on the results of electron microscopy.

In recent years, immunohistochemical features of rhabdomyosarcoma have been studied. Detection of myoglobin antibodies to muscle proteins, desmin-muscle-specific and vimetin-mesenchymal intermediate filaments facilitates diagnosis and allows to clarify the prognosis of the disease. It has been shown that myoglobin-containing rhabdomyosarcoma is more sensitive to chemotherapy than desmin-containing.

The favorite localization of the tumor is the upper inner quadrant of the orbit, therefore the muscle that raises the upper eyelid and the upper rectus muscle are involved in the process early. Ptosis, limited eye movements, its downward and downward inward displacement are the first signs that are noticed by both the patients themselves and those around them. In children, exophthalmos or displacement of the eye with tumor localization in the anterior part of the orbit develops within a few weeks. In adults, the tumor grows more slowly, within a few months. 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 - congestion of the optic nerve. Primarily developing near the upper inner wall of the orbit, the tumor quickly destroys the adjacent thin bone wall, grows into the nasal cavity, causing nosebleeds. Ultrasound scanning, computer tomography, thermography and fine-needle aspiration biopsy are the optimal diagnostic complex of instrumental methods of examination in rhabdomyosarcoma. Treatment is combined. The treatment protocol provides for preliminary polychemotherapy for 2 weeks, after which external irradiation of the orbit is performed. After combined treatment, 71% of patients live for more than 3 years.

Malignant orbital lymphoma

Malignant lymphoma (non-Hodgkin's) has become one of the most common primary malignant tumors of the orbit in recent years. In the orbit, the tumor often develops against the background of autoimmune diseases or immunodeficiency states. Men get sick 2.5 times more often. The average age of patients is 55 years. Currently, malignant non-Hodgkin's lymphoma is considered a neoplasm of the immune system. The process mainly involves lymphoid elements of T- and B-cell affiliation, as well as elements of zero populations. During morphological examination, low-grade B-cell lymphoma is most often diagnosed in the orbit, which contains fairly mature tumor and plasma cells. As a rule, one orbit is affected. Sudden onset of painless exophthalmos, often with a shift of the eye to the side, and edema of the periorbital tissues are characteristic. Exophthalmos can be combined with ptosis. The process progresses steadily locally, red chemosis develops, repositioning of the eye becomes impossible, changes occur in the fundus, most often congestion of the optic nerve. Vision decreases sharply. During this period, pain may appear in the affected orbit.

Diagnosis of malignant orbital lymphoma is difficult. Of the instrumental methods of examination, ultrasound scanning, computed tomography and fine-needle aspiration biopsy with cytological examination are more informative. An examination by a hematologist is necessary to exclude systemic lesions.

External irradiation of the orbit is a virtually uncontested, highly effective method of treating malignant orbital lymphoma. Polychemotherapy is used in case of systemic lesions. The effect of treatment is manifested in the regression of symptoms of the pathological process in the orbit and restoration of lost vision. The prognosis for life and vision in primary malignant lymphoma is favorable (83% of patients survive a 5-year period).

The prognosis for life is sharply worsened in disseminated forms, but the frequency of orbital damage in the latter is slightly more than 5%.