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Pleomorphic rhabdomyosarcoma

ORPHA:293199· ICD-10 C49.9

Definition

A rare soft tissue sarcoma characterized by a high-grade lesion occurring almost exclusively in adults, composed of bizarre polygonal, round, and spindle cells with evidence of skeletal muscle differentiation. Patients usually present with a rapidly growing, painful mass located in the deep soft tissues of the extremities, but also other anatomic regions. Prognosis is generally poor.

Prevalence
<1 / 1 000 000
Inheritance
Not applicable
Age of onset
Adolescent, Adult, Childhood, Elderly, Infancy