Congenital Primary Cutaneous Rhabdomyosarcoma of the Perineum with Mixed Histopathological Features and Unusual Expression of Smooth Muscle Actin: Report of a Case

Authors: Shabaka FH, Abd-Alwadood FA, Moftah NH, Abdel-Halim MRE*, El-Nabarawy EA and Kamel AM

DOI: 10.23880/cdoaj16000109

Volume 2, Issue 1

Abstract

Rhabdomyosarcoma (RMS) affecting the soft tissue of the perianal and perineal regions in children is uncommon and account for 2% of all rhabdomyosarcomas. Primary cutaneous presentation in this anatomical location is extremely rare. We report a 9-month-old female infant with a congenital primary cutaneous RMS presenting as a polypoidal exophytic mass in the perineum mimicking giant condyloma acuminatum (Buschke and Löwenstein tumor). Histopathological examination revealed a predominantly alveolar RMS with areas showing embryonal and pleomorphic features. In addition to expressing desmin and myogenin, the tumor cells also expressed smooth muscle actin.

Keywords: Rhabdomyosarcoma; Soft Tissue; Visceral Organs; Tumors; Embryonal

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