Retroperitoneal spindle cell variant of embryonal rhabdomyosarcoma in a child

Abstract

Spindle cell rhabdomyosarcoma (RMS) is a rare variant of embryonal RMS that has a predilection for young males and most commonly involves the paratesticular region followed by the head and neck. Histopathology reveals elongated spindle cells with fusiform to cigar-shaped nuclei and indistinct eosinophilic cytoplasm arranged in fascicles or whorls. Immunohistochemical workup demonstrates sarcomeric differentiation with reactivity for desmin, myogenin, and MyoD1 markers. Compared with other subtypes, the spindle cell variant in children is associated with a favorable outcome; however, in the adult population, there does not appear to be any prognostic advantage. We are presenting here a case of an embryonal variant of the spindle cell RMS in a 30-month-old male child.

Keywords

• Embryonal
• retroperitoneal
• rhabdomyosarcoma
• spindle