DOI: 10.36347/sjams.2019.v07i09.010

Pages: 2962-2967

Rhabdomyosarcoma (RMS) accounts for <3% of soft tissue sarcoma in adults, but is the most frequent soft tissue sarcoma in children. We performed a retrospective analysis of adolescents and adult patients (≥15 years) with genitourinary RMS treated at our Institute during 1995 – 2015. Among a total of 126 patients with RMS (≥15 years), 26 (20.6%) had genitourinary primary. Twenty were males and 6 were females, with a median age of 17 years. Patients presented with swelling, pain, bleeding / discharge per vagina, urinary retention and renal failure. Among males, primary sites were testis/paratestis in 15, prostate in 3 and urinary bladder in 2. Among females, primary sites were vulva/vagina in 5 and cervix in 1. Eleven had embryonal RMS (ERMS), 7 had alveolar RMS (ARMS), 6 had RMS not otherwise specified (RMS-NOS) and 2 had pleomorphic RMS. Eleven patients were in group I, none in II, 10 in III, and 5 in group IV. Five patients presented with metastasis. All patients received systemic chemotherapy with vincristine, actinomycin and cyclophosphamide (VAC); 12 patients received an additional drug (doxorubicin, cisplatin and/or etoposide). Complete excision was done in 11 patients (orchidectomy in 10 and wide excision in 1), partial cystectomy in 1and biopsy in 14. Eleven patients received local radiotherapy (median dose 40Gy). Four patients progressed, at a median of 21.5 months; 2 each had locoregional and systemic sites of failure. Five year overall survival (OS) was 60%. The 5 year OS for patients with histological subtypes ERMS, RMS-NOS and ARMS was 75%, 50% and 40% respectively. Among the two patients with Pleomorphic RMS, one is still alive at 120 months follow up. Median OS of Group 1 was 70 months and that for combined group 3 and 4 was 25 months (p – 0.215).