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Pleomorphic Rhabdomyosarcoma : Intro

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Pleomorphic Rhabdomyosarcoma (PRMS) - Introduction: PRMS is usually seen in adults over the age of 45 years with a peak incidence in the sixth decade. It is extremely rare in children. It is more common in males. The usual location is skeletal muscles of the lower extremities, especially the thigh. Less common sites of involvement include retroperitoneum, chest or abdominal wall, paratesticular structures, and upper extremities.

The patients usually present with a rapidly growing painless mass of several months' duration. Grossly, it appears as a fleshy, well-circumscribed mass located deep within the skeletal muscles of the extremity. Foci of hemorrhage and necrosis are frequent.

Clinical History: This PRMS presented as a 22 cm mass invading deep soft tissues of thigh in a 65 y/o female. The cut surface shows a multinodular soft, yellow-gray, fleshy tumor with areas of cystic degeneration, hemorrhage, and necrosis. The tumor was composed of spindle cells with abundant cytoplasm, bizarre tumor giant cells with eosinophilic macronucleoli, and strap cells.

Case courtesy of: Prof. Sanjay D. Deshmukh, Dept. of Pathology, Smt. Kashibai Navale Medical College, Pune, India.

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