Rhabdomyosarcoma of the eye

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

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Symptoms of Rhabdomyosarcoma of the Eye

Rhabdomyosarcoma of the eye manifests itself in the first decade of life (on average at 7 years) in the form of rapidly progressing exophthalmos, which may resemble an inflammatory process.

• The tumor is often located retrobulbar, less often in the upper and lower parts of the orbit.
• A palpable mass and ptosis are found in approximately 1/3 of patients.
• Swelling and injection of the skin covering the tumor develop later, but without an increase in its temperature.
• Parameningeal tumors cause bone destruction, disseminate to the lymph nodes and affect the central nervous system.

CT reveals a formation with unclear boundaries of homogeneous density, often with destruction of the adjacent bone. In advanced cases, invasion into the paranasal sinuses may be observed.

General workup to detect metastases includes chest x-ray, liver function tests, bone marrow biopsy, lumbar puncture, and skeletal survey. Common sites of metastasis are the lungs and bones.

Differential diagnosis of rhabdomyosarcoma of the eye

1. Orbital cellulitis is characterized by similar acute symptoms, but with rhabdomyosarcoma, unlike cellulitis, the skin temperature does not increase.
2. Granulocytic sarcoma may also present as a rapidly growing orbital mass.

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Treatment of rhabdomyosarcoma of the eye

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