Primitive neuroectodermal tumour of kidney: an unusual entity
Keywords:
Primitive Neuroectodermal Tumour, Anaplastic, Neuron Specific Enolase, Carcinoid Tumour
Abstract
Primitive neuroectodermal tumor (rPNET ) of kidney is an extremely rare tumour which occurs in children and young adults and has only a few published reports. The present case is of a 24-year-old female presenting with complaining of pain abdomen since 2-3 months duration. USG guided FNAC showed a cellular aspirate composed of small round to oval cells with scanty or small amount of cytoplasm was seen. Cells were arranged in clusters with occasional microacinar or papillary arrangement was also noted. Two possibilities were suggested- 1.Carcinoid tumour, 2. small cell anaplastic carcinoma. Histopathological and immunohistochemical (IHC) correlation was done. A highly malignant small cell neoplasm composed of small round cells having scanty cytoplasm and dense nuclei against hemorrhagic background was seen. IHC panel was performed and was found to be strongly positive for MIC-2 (CD99) and showed weak positivity for NSE and ki -67. A final diagnosis of Primitive Neuro-ectodermal Tumour of kidney was made.References
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2. Pomara G, Cappello F, Cuttano MG, Rappa F, Morelli G, Mancini P. Primitive neuroectodermal tumor of the kidney: case report. BMC cancer 2004;4:3
3. Mandal PK, Mukherjee S, Roy S, Bhattacharyya NK. PNET of kidney: Report of 4 cases. Indian J Med Paediatr Oncol. 2012; 33:130-3
4. Habermann H, Beneschb M, Schipsa L, Pummera K, Ratschekc M, Uggowitzerd MM, et al. Findings and clinical course of localized primitive peripheral neuroectodermal tumor of the kidney. Urol Int 2003,71:319-21
5. Parham D, Roloson G, Feely M, Green DM, Bridge JA, Beckwith JB. Primary malignant neuroepithelial tumors of the kidney: clinicopathologic analysis of 146 adult and pediatric cases from the National Wilms’ tumor study group pathology center. Am J Surg Pathol 2001, 25:133–146.
6. Wu Y, Zhu Y, Chen H, Huang Y, Wei Q, Chen H, et al. Primitive neuroectodermal tumor of the kidney with inferior vena cava tumor thrombus during pregnancy. Chin Med J 2010, 123:2155–8.
7. Eble JN, Sauter G, Epstein JI: (Eds): Pathology and genetics of tumors of the urinary system and male genital organs. Lyon: IARC Press; 2004.
Published
2015-05-10
Issue
Section
Case Report
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