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E-JCRT CORRESPONDENCE
Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1038

Fulminant Buddchiari syndrome caused by renal primitive neuroectodermal tumor with inferior vena cava thrombus extending to atrium


1 Department of Urology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Nuclear Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Pathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Correspondence Address:
Dig Vijay Singh
Department of Urology, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.139528

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Primitive neuroectodermal tumors (PNET) of the kidney are rare, the diagnosis usually being made at histopathology. A young female presented with a massive right renal mass with features of hepatic dysfunction. Computed tomography scan of the abdomen revealed a large tumor of right kidney with tumor thrombus extending from inferior vena cava (IVC) to right atrium with features suggesting buddchiari syndrome (BCS). Needle biopsy of mass showed a round cell neoplasm and positive staining for neuron specific enolase and minimum inhibitory concentration-2 on immunohistochemistry. She was managed with neo-adjuvant chemotherapy, surgery and adjuvant chemotherapy. To the best of our knowledge this is the first case of renal PNET with inferior IVC tumor thrombus extending to right atrium with BCS. We suggest that renal PNET should be kept in mind as a differential diagnosis in young adults presenting with a large kidney mass extending to IVC that shows evidence of necrosis on imaging, which may be associated with BCS as in index case.


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