PNET of kidney : Report of four cases

 

PDF Download Permissions and Reprints

Abstract

Primitive neuroectodermal tumor (PNET) of kidney is a rare tumor of kidney with only a few published reports. We report here four cases of PNET of kidney in the age group between 30 and 50 years who had complaints of vague pain and lump in loin. Hematuria was present in one case. Imaging of all cases revealed renal mass. The pathologic findings were consistent with PNET in all cases-confirmed by immunohistochemistry with diffuse membrane positivity of tumor cells of CD99. We could not do fluorescent in situ hybridization to demonstrate EWS-FLI-1 gene fusion. Each case was in the advanced stage. However, after giving postoperative radiotherapy and chemotherapy patients are still alive. Reporting of these cases are important as we got them in a short span of 3 years. In view of its poor prognosis, aggressive nature and different therapeutic approach- renal PNET should be differentiated from other small blue round cell tumors like neuroblastoma, rhabdoid tumor of kidney, nephroblastoma, small cell carcinoma, synovial sarcoma (monophasic, poorly differentiated) and non-Hodgkin lymphoma (NHL) by immunohistochemistry, cytogenetic, and molecular genetics study to see the different gene rearrangements in NHL and 3p deletion in small cell carcinoma.

Publication History

Article published online:
13 April 2022

© 2012. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

• References

• 1 Marley EF, Liapis H, Humphrey P, Nadler RB, Siegel CL, Zhu X, et al. Primitive neuroectodermal tumor of the kidney - another enigma : A0 pathologic, immunohistochemical, and molecular diagnostic study. Am J Surg Pathol 1997;21:354-9.
• 2 Jimanez RE, Folpe AL, Lapham RL, Ro Jy, O′shea PA, Weiss SW, et al. Primary Ewing′s sarcoma/primitive neuroectodermal tumor of the kidney : A0 clinicopathologic and immunohistochemical analysis of 11 cases. Am J Surg Pathol 2002;26:320-7.
• 3 Quezedo M, Benjamin DR, Tsokos M. EWS/FLI-1 fusion transcripts in three peripheral primitive neuroectodermal tumors of the Kidney. Hum Pathol 1997;28:767-71.
• 4 Sheaff M, Mcmanus A, Scheimberg I, Paris A, Shipley J, Baithun S. Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization. Am J Surg Pathol 1997;21:461-8.
• 5 Parham DM, Roloson GJ, Feelay M, Green DM, Bridge JA, Beckwith JB. Primary Malignant Neuroepithelial tumors of the kidney : A0 clinicopathologic analysis of 146 adult and pediatric cases from the National Wilms′ Tumor Study Group Pathology Center. Am J Surg Pathol 2001;25:133-46.
• 6 Castella R, Moch H, Rochiltz C, Meier V, Seifert B, Mihatsch MJ, et al. Metastatic Primitive neuroectodermal tumor of the kidney in adults. Eur Urol 2001;39:613-7.
• 7 Weeks DA, Beckwith JB, Mieran GW, Zuppan CW. Renal neoplasms mimicking rhabdoid tumor of kidney. A report from the National Wilms′ Tumor Study Pathology Center. Am J Surg Pathol 1991;15:1042-54.
• 8 Rodriguez-Galindo C, Marina NM, Fletcher BD, Parham DM, Bodner SM, Meyer WH. Is primitive neuroectodermal tumor of kidney a distinct entity? Cancer 1997;79:2243-50.
• 9 Reya T, Morrison JS, Clarke MF. Stem cells, Cancer and cancer stem cells. Nature 2001;414:105-11.