Primary Ewing sarcoma/Primitive neuroectodermal tumor of the kidney: an aggressive neoplasm mimicking Wilms tumor

Dr.Smrita Singh; Dr. Premalata Chennagiri S; Dr. Kavitha B.L.; Dr. Prasanna Kumari; Dr. Madhu S.D.; Dr. Rekha V. Kumar

Volume : VI, Issue : III, March - 2017

Primary Ewing sarcoma/Primitive neuroectodermal tumor of the kidney: an aggressive neoplasm mimicking Wilms tumor

Dr. Smrita Singh, Dr. Premalata Chennagiri S, Dr. Kavitha B. L. , Dr. Prasanna Kumari, Dr. Madhu S. D. , Dr. Rekha V. Kumar

Abstract :

Ewing sarcoma/Primitive neuroectodermal tumors (ES/PNET) constitute a family of neoplasms of presumed neuroectodermal origin most often presenting as bone or soft tissue masses. These neoplasms can rarely affect the kidney mimicking Wilms tumor. There are few reported case series and scattered case reports of ES/ PNET of the kidney in the world literature. Six cases of primary renal ES/PNET were retrieved from the histopathology records at our institute over a period of three years and the pathology and immunohistochemical slides reviewed along with the cytogenetic analysis/fluorescence in–situ hyidization (FISH) reports and the clinical details. The diagnosis was confirmed for the presence of t(11;22) either by karyotyping or FISH (EWSR1) the hallmark of this family of tumors. The diagnosis of primary renal Ewing sarcoma/PNET can be difficult and often requires confirmation by cytogenetics or FISH in addition to immunohistochemistry.