title: Primary Paediatric Renal Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature.
abstract: 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.
authors.affiliation: Department of Pathology, Nil Ratan Sircar Medical College & Hospital, KOLKATA, INDIA.
authors.forename: Binny
authors.initials: B
authors.lastname: Khandakar
authors.forename: Moumita
authors.initials: M
authors.lastname: Maiti
authors.forename: Soumit
authors.initials: S
authors.lastname: Dey
authors.forename: Prasenjit Sen
authors.initials: PS
authors.lastname: Ray
authors.forename: Palas
authors.initials: P
authors.lastname: Bhattacharyya
authors.forename: Ranu
authors.initials: R
authors.lastname: Sarkar
mesh_terms: D002648:Child; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000473:pathology*; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000473:pathology*