title: Primitive neuroectodermal tumor/Ewing's sarcoma in adult uro-oncology: A case series from a developing country.
abstract: Peripheral primitive neuroectodermal tumor/Ewing's sarcoma (PNET/EWS) is primarily a tumor of soft tissues and bones. Primary localization of PNET/EWS in genitourinary organs is rare. No data on this localization of PNET/EWS are available in literature from Pakistan. We searched our adult uro-oncology records from 1994 till date and identified all cases of adult genitourinary and adrenal PNET/EWS diagnosed on histology and immunohistochemistry. Their case records were reviewed to obtain data on demographics, presentation, pathologic features, management and outcome. Six cases were found; all were young and had aggressive disease at presentation. Four had renal PNET/EWS. One case each of prostate and adrenal PNET/EWS was seen. Surgery and chemotherapy formed the mainstay of management. Three patients (50%) died during treatment, two were lost to follow-up and one case with renal PNET/EWS showed good initial response to chemotherapy but was later on lost to follow-up. In conclusion, PNET/EWS should be considered in the differential diagnosis of genitourinary malignant tumors in young patients. These tumors are aggressive with poor outcome.
authors: Department of Urology, Sindh Institute of Urology and Transplantation (SIUT), Civil Hospital, Karachi, Pakistan.
authors: Rehan
authors: R
authors: Mohsin
authors: Altaf
authors: A
authors: Hashmi
authors: Muhammed
authors: M
authors: Mubarak
authors: Gohar
authors: G
authors: Sultan
authors: Asad
authors: A
authors: Shehzad
authors: Abdul
authors: A
authors: Qayum
authors: Syed Ali Anwer
authors: SA
authors: Naqvi
authors: Syed Adeeb Ul Hassan
authors: SA
authors: Rizvi