Indian Journal of Pathology and Microbiology
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Year : 2014  |  Volume : 57  |  Issue : 2  |  Page : 298-300

Primary primitive neuroectodermal tumor of kidney: A rare case report with diagnostic challenge

Department of Pathology and Laboratory Science, Armed Forces Medical College, Command Hospital, Pune, Maharashtra, India

Correspondence Address:
Devika Gupta
Department Of Pathology and Laboratory Science, Armed Forces Medical College, Command Hospital, Pune - 411 040, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.134723

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Primary primitive neuroectodermal tumors (PNETs) of the kidney are quite rare and can be mistaken for a wide variety of other small round blue cell tumors which includes rhabdomyosarcoma, Wilm's tumor, carcinoid, neuroblastoma, clear cell sarcoma of the kidney, lymphoma etc. Renal Ewings/PNET can occur in the age group from 4 to 61 years. Approximately, 90% of Ewing sarcoma (ES)/PNET have a specific t(11;22) which results in a chimeric EWS-FLI-1 fusion protein. Immunohistochemical for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. Herein, we have an interesting presentation in a 23-year-old male who came with neck pain and progressive quadriparesis and was diagnosed as a case of poorly differentiated malignant tumor with a differential of lymphoma versus metastatic renal cell carcinoma. The patient's condition deteriorated fast and he had a rapid downhill course. The final diagnosis of Ewings/PNET was confirmed at autopsy.

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