Turkish Journal of Pathology

Türk Patoloji Dergisi

Turkish Journal of Pathology

Turkish Journal of Pathology

2018, Vol 34, Num, 3     (Pages: 251-254)

Primary Paediatric Renal Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature

Binny KHANDAKAR 1, Moumita MAİTİ 1, Soumit DEY 1, Prasenjit Sen RAY 1, Palas BHATTACHARYYA 1, Ranu SARKAR 1

1 Department of Pathology, Nil Ratan Sircar Medical College & Hospital, KOLKATA, INDIA

DOI: 10.5146/tjpath.2015.01340
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Ewing sarcoma/primitive neuroectodermal tumor (PNET) is a high-grade malignant neoplasm commonly affecting bones of the thoracic region. Primary Ewing sarcoma/PNET of the kidney is exceptional; it commonly affects young adults and is rarely reported in children.

Here we describe a case of renal PNET in a 10-year-old girl who presented at our institute with haematuria and abdominal lump. Computer tomography scan revealed a huge mass in the right kidney. Computer tomography-guided aspiration from the mass showed cytomorphological features of a small blue round cell tumor. The patient underwent right radical nephrectomy. Histopathology with supplementary immunohistochemistry confirmed the diagnosis of PNET. She has been receiving treatment with adjuvant chemotherapy post-surgery and is currently disease free.

Primary renal PNET is a distinct and rare entity. This tumor is very aggressive with low survival rate, even with a multi-disciplinary approach. We reported the case because it is rare in children, poses diagnostic challenges, is aggressive in behaviour and responds poorly to treatment.

Keywords : Ewing sarcoma, Kidney, Primitive neuroectodermal tumor