Shatakshee Tewari

Department of Pathology, Autonomous State Medical College, Hardoi, Uttar Pradesh, India

Varsha Kumar

Department of Pathology, Moti Lal Nehru Medical College, Prayagraj, Uttar Pradesh, India

Vatsala Misra

Department of Pathology, Moti Lal Nehru Medical College, Prayagraj, Uttar Pradesh, India

Keywords

child, cytology, renal, pnet, diagnosis

Abstract

Renal sarcomas are rare, accounting for less than 1% of all renal malignancies. Ewing sarcoma/PNET of the kidney is an aggressive and extremely rare neoplasm with only 120 cases reported so far. It is seen in young adults, and only few pediatric cases have been reported so far. We report an 8-year-old boy presenting with progressive left lumbar swelling for 1 year and a prior history of antitubercular therapy. Examination revealed a tender subcutaneous abscess measuring 7×5 cm in the left lumbar region. Fine-needle aspiration cytology suggested a small round blue cell tumor. Histopathological examination, supported by immunohistochemistry, confirmed the diagnosis of renal PNET, which is highly aggressive as compared to PNET arising from other sites. It needs to be distinguished from other primary renal tumors owing to its poor prognosis and aggressive nature. Clinical and radiographic features are nonspecific leading to diagnostic challenges. Definitive diagnosis requires histopathological examination and IHC.

References

1. Risi E, Iacovelli R, Altavilla A, et al. Clinical and pathological features of primary neuroectodermal tumor/Ewing sarcoma of the kidney. Urology. 2013;82:382–386. https://doi.org/10.1016/j.urology.2013.03.024
2. Suzuki I, Kubota M, Murata S, Makita N, Tohi Y, Kawakita M. A case of Ewing sarcoma family tumor of the kidney treated with robotic-assisted partial nephrectomy. Urology Case Reports. 2019;25:100900. https://doi.org/10.1016/j.eucr.2019.100900
3. Asiri M, Al-Sayyad A. Renal primitive neuroectodermal tumour in childhood: case report and review of literature. Canadian Urological Association. 2010;4(6):E158–E160. https://doi.org/10.5489/cuaj.969
4. Almeida MF, Patnana M, Korivi BR, Kalhor N, Marcal L, Lall C, et al. Ewing sarcoma of the kidney: A rare entity. Case Rep Radiol. 2014;2014:283902. https://doi.org/10.1155/2014/283902
5. Sharifi Doloui D, Fakharian T, Yahyavi V, Nekooei S, Zivarifar HR, Ghafarzadegan K. Primitive neuroectodermal tumor with kidney involvement: a case report. Iranian Journal of Radiology. 2014;11(2):e4661. https://doi.org/10.5812/iranjradiol.4661
6. Rahmani K, Taghipour Zahir S, Yazdi MB, et al. A rare case of primary Ewing’s sarcoma presenting in the posterior nasal cavity with extension into the sphenoid sinus and a review of the literature. Otolaryngology Case Reports. 2018;6:34–37. https://doi.org/10.1016/j.xocr.2018.01.003
7. Mandal PK, Mukherjee S, Roy S, Bhattacharyya NK. PNET of kidney: report of four cases. Indian Journal of Medical and Paediatric Oncology. 2012;33:130–133. https://doi.org/10.4103/0971-5851.99754
8. Mor Y, Nass D, Raviv G, et al. Malignant peripheral primitive neuroectodermal tumor (PNET) of the kidney. Med Pediatr Oncol 1994; 23:437–440.
9. Moch H, Humphrey PA, Ulbright TM, Reuter VE, eds. WHO classification of tumours of the urinary system and male genital organs. 4th ed. Lyon, France: IARC Press; 2016.
10. Khandakar B, Maiti M, Dey S, Ray PS, Bhattacharyya P, Sarkar R. Primary paediatric renal primitive neuroectodermal tumor: a case report and review of the literature. Turkish Journal of Pathology. 2018;34(3):251–254. https://doi.org/10.5146/tjpath.2015.01340
11. Aghili M, Rafiei E, Mojahed M, Zare M. Renal primitive neuroectodermal tumor: does age at diagnosis impact outcomes? Rare Tumors. 2012;4:e15. https://doi.org/10.4081/rt.2012.e15
12. Parham DM, Roloson GJ, Feely M, et al. Primary malignant neuroepithelial tumors of the kidney: a clinicopathologic analysis of 146 adult and pediatric cases from the National Wilms’ Tumor Study Group Pathology Center. American Journal of Surgical Pathology. 2001;25:133–146. https://doi.org/10.1097/00000478-200102000-00001
13. Bartholow TL, Parwani AV. Renal primitive neuroectodermal tumors. Archives of Pathology & Laboratory Medicine 2012;136(6):686–690. https://doi.org/10.5858/arpa.2011-0104-RS
14. Narayanan G, Rajan V, Preethi TR. Primitive neuroectodermal tumors of the kidney. Proceedings (Baylor University Medical Center). 2017;30(2):205–208. https://doi.org/10.1080/08998280.2017.11929588
15. Shet T, Viswanathan S. The cytological diagnosis of paediatric renal tumours. Journal of Clinical Pathology. 2009;62:961–969. https://doi.org/10.1136/jcp.2009.064659