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

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 c...

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Published inIndian journal of pathology & microbiology Vol. 57; no. 2; pp. 298 - 300
Main Authors Kakkar, Sunita, Gupta, Devika, Kaur, Gurpreet, Rana, Vandana
Format Journal Article
LanguageEnglish
Published India Medknow Publications and Media Pvt. Ltd 01.04.2014
Wolters Kluwer Medknow Publications
Subjects
12E7 Antigen
Antigens, CD - analysis
Case studies
Cell Adhesion Molecules - analysis
Diagnosis
Diagnosis, Differential
Fatal Outcome
FLI-1
Histocytochemistry
Humans
Immunohistochemistry
kidney
Kidney diseases
Kidney Neoplasms - diagnosis
Kidney Neoplasms - pathology
Male
Microfilament Proteins - analysis
Microscopy
Neuroectodermal Tumors, Primitive - diagnosis
Neuroectodermal Tumors, Primitive - pathology
primitive neuroectodermal tumor
Radiography, Abdominal
Radiography, Thoracic
Receptors, Cytoplasmic and Nuclear - analysis
Tomography, X-Ray Computed
Young Adult
India
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Summary: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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ISSN:0377-4929
0974-5130
DOI:10.4103/0377-4929.134723