Primitive neuroectodermal tumor/Ewing′s sarcoma in adult uro-oncology: A case series from a developing country

• Published in: Urology annals 2011, Vol.3 (2), p.103-107
• Main Authors: Mohsin, Rehan, Hashmi, Altaf, Mubarak, Muhammed, Sultan, Gohar, Shehzad, Asad, Qayum, Abdul, Naqvi, Syed AliAnwer, Rizvi, SyedAdeeb ul Hassan
• Format: Article
• Language: English
• Subjects: Adrenal gland; kidney; primitive neuroectodermal tumor/Ewing′s sarcoma; prostate; uro-oncology
• ISSN: 0974-7796; 0974-7834
• DOI: 10.4103/0974-7796.82180

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.