Primary Paediatric Renal Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature
| Autor: | Binny KHANDAKAR, Moumita MAİTİ, Soumit DEY, Prasenjit Sen RAY, Palas BHATTACHARYYA, Ranu SARKAR |
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| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: | |
| Zdroj: | Türk Patoloji Dergisi, Vol 34, Iss 3, Pp 251-254 (2018) |
| Druh dokumentu: | article |
| ISSN: | 1018-5615 1309-5730 |
| DOI: | 10.5146/tjpath.2015.01340 |
| Popis: | 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. |
| Databáze: | Directory of Open Access Journals |
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