| CASE REPORT |
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| Year : 2014 | Volume
: 57
| Issue : 2 | Page : 298-300 |
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Primary primitive neuroectodermal tumor of kidney: A rare case report with diagnostic challenge
Sunita Kakkar, Devika Gupta, Gurpreet Kaur, Vandana Rana
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
India
 Source of Support: None, Conflict of Interest: None  | Check |
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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