Malignant neoplasm of orbit

Tumors of the orbit make up 23-25% of all neoplasms of the organ of vision. In it, virtually all tumors that occur in humans develop. The incidence of primary tumors is 94.5%, secondary and metastatic tumors - 5.5 %.

Primary malignant tumors of orbit make up no more than 0.1% of all malignant tumors of the person, at the same time in the group of all primary tumors - 20-28%. They practically with the same frequency develop in all age groups. Men and women get sick the same way. Malignant tumors of the orbit, as well as benign, are polymorphic in histogenesis, the degree of their malignancy is variable. More common sarcomas and cancer. The common clinical symptoms of malignant tumors of the orbit are early-arising diplopia and edema of the eyelids, originally transient, appearing in the morning, and then passing into a stationary one. These tumors are characterized by a constant pain syndrome, exophthalmos occur early and grows rapidly enough (within a few weeks or months). As a result of compression of the neurovascular bundle by a growing tumor, deformity of the eye and large exophthalmos, the protective function of the eyelids is broken: in these patients, dystrophic changes in the cornea, which result in complete melting, rapidly increase.

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

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Primary cancer of the orbit

Primary cancer of the orbit is rare. Men are sick more often. Primary cancer of the orbit is considered to be the result of a tumor transformation of the dystopic epithelial cells into the 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 in the direction opposite to the localization of the tumor, its mobility is sharply limited, the exophthalmos slowly increase, which never reaches higher degrees, but the reposition of the eye is impossible. A dense infiltrative growing tumor squeezes the venous pathways of the orbit, resulting in stagnation in the episcleral veins, an increase in the ophthalmotonus. The eye is as if immured by a tumor in the orbit, its bony edges are "smoothed out" by the surrounding tumor and become not available for palpation. The visual functions are preserved for a long period of time, despite the secondary intraocular hypertension.

The initial growth of the tumor at the apex of the orbit is manifested by early pains in it with irradiation into the corresponding half of the head and by diplopia. As the tumor grows, full ophthalmoplegia occurs. The primary atrophy of the optic nerve disk with a rapid decrease in visual functions is characteristic. Exophthalmos are noticed late, usually it does not reach high degrees.

The features of the clinical picture and the results of ultrasound scanning allow us to determine the infiltrative spread of the pathological process without revealing its nature. Computed tomography demonstrates a dense infiltrative growing tumor, its relationship with the surrounding soft tissue and bone structures of the orbit. The results of radionuclide scintigraphy and thermography testify to the malignant nature of tumor growth. A fine needle aspiration biopsy with a cytological examination of the obtained material is possible when the tumor is located in the anterior part of the orbit. If it is located at the apex of the orbit, aspiration is accompanied by the danger of damage to the eye.

Surgical treatment (subperiosteal exenteration of the orbit) is indicated with anterior localization of the process. Treatment of more deeply located tumors is difficult, since they early germinate into the cavity of the skull. Radiation therapy is ineffective, since the primary heterotopic cancer is refractory to irradiation. The forecast is poor. At the front location, the tumor metastasizes into the regional lymph nodes. Cases of hematogenous metastasis are unknown, but with the early germination of the tumor into the underlying bone and skull cavity, the process becomes almost uncontrollable.

Sarcomas are the most frequent cause of primary malignant growth in orbit. They constitute 11-26% of all malignant tumors of the orbit. The source of the development of the sarcoma in orbit can be almost any tissue, but the frequency of occurrence of its individual species is different. Age of patients from 3-4 weeks to 75 years.

Rhabdomyosarcoma of the orbit

Rhabdomyosarcoma of the orbit - an extremely aggressive tumor of the orbit, is the most common cause of malignant growth in the orbit in children. Boys are ill almost 2 times more often. The source of growth of rhabdomyosarcoma 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, after 5 years - the alveolar type. As a rule, rhabdomyosarcoma consists of several types of elements (mixed version). An accurate diagnosis can be established only on the basis of the results of electron microscopy.

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

The preferred location of the tumor is the upper quadrant of the orbit, so the muscle that lifts the upper eyelid and the upper rectus muscle are involved early in the process. Ptosis, limitation of eye movements, displacement of it downwards and downwards are the first signs, which are noticed by the patients themselves and by the surrounding people. In children, exophthalmos or eye displacement when the tumor is localized in the anterior part of the orbit develops within a few weeks. In adults, the tumor grows more slowly, for several months. The rapid increase in exophthalmos is accompanied by the appearance of stagnant changes in the episcleral veins, the eye gap does not completely close, infiltrates on the cornea and its ulceration are noted. On the fundus there is a stagnant disk of the optic nerve. Primarily developing near the upper inner wall of the orbit, the tumor quickly destroys the adjacent thin bone wall, sprouts into the nasal cavity, causing nasal bleeding. Ultrasound scanning, computer tomophage, thermophage and fine needle aspiration biopsy is the optimal diagnostic complex of instrumental research methods for rhabdomyosarcoma. Treatment combined. The protocol of treatment provides for preliminary polychemotherapy for 2 weeks, after which external irradiation of the orbit is performed. After combined treatment for more than 3 years, 71% of patients live.

Malignant orbit lymphoma

Malignant lymphoma (non-Hodgkin's) in frequency in recent years is one of the first places among primary malignant tumors of the orbit. In the orbit, the tumor often develops against the background of autoimmune diseases or immunodeficiency states. Men are sick 2.5 times more often. The average age of the patients is 55 years. Currently, malignant non-Hodgkin's lymphoma is considered as a neoplasm of the immune system. The process involves mainly lymphoid elements of T- and B-cell identity, as well as elements of zero populations. At morphological research in an orbit more often diagnose B-cellular lymphoma of a low degree of malignancy in which enough mature tumor and plasma cells contain. As a rule, one orbit is affected. Characterized by the sudden appearance of painless exophthalmos, more often with an eye shift to the side, and swelling of periorbital tissues. Exophthalmos can be combined with ptosis. The process locally progresses steadily, red chemosis develops, the reposition of the eye becomes impossible, changes occur on the fundus, more often the stagnant disk of the optic nerve. Sharply reduced vision. During this period, pain may occur in the affected orbit.

The diagnosis of malignant lymphoma of the orbit is difficult. From instrumental methods of research, ultrasound scanning, computed tomography and fine needle aspiration biopsy with cytological examination are more informative. It is necessary to examine the hematologist to exclude systemic damage.

External irradiation of the orbit is practically a non-alternative highly effective method of treating malignant lymphoma of the orbit. Polychemotherapy is used in systemic lesions. The effect of treatment is manifested in the regression of the 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 experience a 5-year period).

The prognosis for life sharply worsens with disseminated forms, but the incidence of orbital damage in the latter is just over 5%.