Rhabdomyosarcoma of the eye

The rhabdomyosarcoma of the eye is the most common primary malignant tumor of the orbit in children. The main role of an ophthalmologist is to establish a diagnosis by biopsy and refer the patient to a pediatric oncologist.

[1], [2], [3], [4]

Symptoms of rhabdomyosarcoma of the eye

The rhabdomyosarcoma of the eye appears in the first decade of life (an average of 7 years) in the form of a rapidly progressing exophthalmos, it may resemble an inflammatory process.

• The tumor is often located retrobulbarno, less often - in the upper and lower parts of the orbit.
• Palpable formation and ptosis are found in about 1/3 of the patients.
• Swelling and injection of the skin covering the tumor develop later, but without increasing its temperature.
• Paraimenialnye tumors cause bone destruction, disseminate into lymph nodes and affect the CNS.

CT scan reveals formation with fuzzy boundaries of homogeneous density, often with destruction of adjacent bone. In far-reaching cases, sprouting can occur in the paranasal sinuses.

A general examination for the detection of metastases includes chest X-ray, liver tests, bone marrow biopsy, lumbar puncture and skeletal examination. Frequent regions of metastasis are lungs and bones.

Differential diagnosis of rhabdomyosarcoma of the eye

1. Cellulite of the orbit is characterized by similar acute symptoms, but with rhabdomyosarcoma, unlike cellulite, skin temperature does not increase.
2. Granulocyte sarcoma can also be a fast-growing orbital formation.

[5], [6], [7], [8]

Treatment of rhabdomyosarcoma of the eye

• Radiation therapy in combination with chemotherapy vincristine, actinomycin and cyclophosphamide.
• Surgical removal is used for rarely relapsing and radioresistant tumors.

The prognosis depends on the stage and localization of the process at the time of diagnosis. Patients with isolated orbital lesions are cured in 95% of cases.