Abstract

Rhabdomyosarcomas form a heterogeneous group of malignant soft tissue tumors characterized by immature striated muscle differentiation. Epithelioid and spindle cells rhabdomyosarcoma is a recently described entity, mainly localized intraosseously and predominantly found in young patients. Its late diagnosis and high aggressiveness confer a grim prognosis to this tumor, highlighting the importance of early recognition and appropriate management. We present herein the clinical, histopathological, immunohistochemical, and molecular aspects of this entity through a case of misleading presentation.

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