Main Article Content
Immunohistochemistry; IVC thrombus; Multimodality treatment; PNET, RENAL PNET; Renal Pelvis PNET
The primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare neoplasm, the diagnosis of which mainly depends upon his-topathology, immunohistochemistry (IHC), and cytogenetics. A handful of cases reported in the literature mention about aggressive features of this neoplasm. The purpose of our study was to review our experience in not only the diagnosis and management of the patients with renal PNET but also to highlight its propensity to involve inferior vena cava (IVC) and also present a rare occurrence of Ewing’s sarcoma (ES)/PNET of the renal pelvis.
The clinical, operative, and histopathology records of four patients of renal PNET treated between January 2017 and December 2019 were reviewed and data analyzed concerning the available literature. Out of the four patients treated, two had level III and IV IVC thrombus, and one had dense desmoplastic adhesions with the IVC wall. One of the cases had a rare presentation of ES/PNET of the renal pelvis. All patients were managed surgically, while only one patient received adjuvant chemotherapy and following up with remission for the last 2 years and 4 months. On IHC, cluster of differentiation-99 (CD-99) was positive in all patients, and three were positive for Friend leukemia integration-1. PNET of the kidney is primarily an immunohistopathological diagnosis. This neoplasm has an increased propensity for the local invasion of surrounding structures. A multimodality approach with surgery, chemotherapy, and radiotherapy could offer better outcomes, although the prognosis of these tumors remains poor.
2. Seemayer TA, Thelmo WI, Bolande RP, Wiglesworth FW. Peripheral neuroectodermal tumors. Perspect Pediatr Pathol.1975;2:151–72.
3. Desai S. Primary primitive neuroectodermal tumor of the uri-nary bladder. Histopathology.1998;32(5):477–8. http://dx.doi. org/10.1046/j.1365-2559.1998.0358a.x
4. Peyromature M, Viellefond A, Boucher E, De Pinieux G, Beuzeboc P, Debré B, et al. Primitive neuroectodermal tumor of the prostate. J Urol. 2003;170(1):182–3. http://dx.doi. org/10.1097/01.ju.0000065880.06201.5c
5. Rose PG, O’Toole RV, Keyhani-Rofagha S, Qualman S, Boutselis JG. Malignant primitive peripheral neuroectodermal tumor of the uterus. J Surg Oncol. 1987;35(3):165–9. http://dx. doi.org/10.1002/jso.2930350305
6. Casella R, Moch H, Rochlitz C, Meier V, Seifert B, Mihatsch MJ, et al. Metastatic primitive neuroectodermal tumor of the kidney in adults. Eur Urol. 2001;39(5):613–17. http://dx.doi. org/10.1159/000052514
7. Chakrabarti N, Dewasi N, Das S, Bandyopadhyay A, Bhaduri N. Primary Ewing’s sarcoma/primitive neuroectodermal tumor of kidney – A diagnostic dilemma. Iran J Cancer Prev. 2015;8(2):129–33.
8. Mohsin R, Hashmi A, Mubarak M, Sultan G, Shehzad A, Qayum A, et al. Primitive neuroectodermal tumor/Ewing’s sarcoma in adult urooncology: A case series from a developing country. Urol Ann. 2011;3(2):103–7. http://dx.doi. org/10.4103/0974-7796.82180
9. Risi E, Iacovelli R, Altavilla A, Alesini D, Palazzo A, Mosillo C, et al. Clinical and pathological features of primary neuroectodermal tumor/Ewing’s sarcoma of the kidney. Urology. 2013;82(2):382–6. http://dx.doi.org/10.1016/j. urology.2013.04.015
10. Sun Q, Miao B, Lao X, Yuan P, Cai J, Zhan H. Primitive neuroectodermal tumor of the kidney at the advanced stage: A case series of eight Chinese patients. Mol Clin Oncol. 2018;8(6):743– 48. http://dx.doi.org/10.3892/mco.2018.1611
11. Narayanan G, Rajan V, Preethi TR. Primitive neuroectodermal tumors of the kidney. Proc (Baylor Univ Med Cent). 2017;30(2):205–8. http://dx.doi.org/10.1080/08998280.2017.119 29588
12. Liu Z, Wang X, Lu Y, Chen L, Lu Y. Primary Ewing sarcoma/ primitive neuroectodermal tumor of the renal pelvis: A case report. World J Surg Oncol. 2014;12:293. http://dx.doi. org/10.1186/1477-7819-12-293
13. Zhou M, Wang Z-C, Shen H, Huang J-W, Ma Z-L. Primary Ewing’s sarcoma of the renal pelvis in an adult: A rare case report and literature review. Int J Clin Exp Med. 2018;11(11):12796–800.
14. Seth A, Mahapatra SK, Nayak B, Saini AK, Biswas B. Primitive neuroectodermal tumors of the kidney: Our experience in a tertiary care center. Indian J Cancer. 2016;53(1):109–12. http://dx. doi.org/10.4103/0019-509X.180854
15. Marley EF, Liapis H, Humphrey PA, Nadler RB, Siegel CL, Zhu X, et al. Primitive neuroectodermal tumor of the kidney— Another enigma: A pathologic, immunohistochemical, and molecular diagnostic study. Am J Surg Pathol. 1997;21(3):354–9. http://dx.doi.org/10.1097/00000478-199703000-00013
16. Parham DM, Roloson GJ, Feely M, Green DM, Bridge JA, Beckwith JB. 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. Am J Surg Pathol. 2001;25(2):133–46. http://dx.doi. org/10.1097/00000478-200102000-00001
17. Bing Z, Zhang P, Tomaszewski JE, Maclennan GT. Primary Ewing sarcoma/primitive neuroectodermal tumor of the kidney. J Urol. 2009;181(3):1341–2. http://dx.doi.org/10.1016/j. juro.2008.12.029
18. Folpe AL, Hill CE, Parham DM, O’Shea PA, Weiss SW. Immunohistochemical detection of FLI-1 protein expression: A study of 132 round cell tumors with emphasis on CD99-positive mimics of Ewing’s sarcoma/primitive neuroectodermal tumor. Am J Surg Pathol. 2000;24(12):1657–62. http://dx.doi. org/10.1097/00000478-200012000-00010
19. Angel JR, Alfred A, Sakhuja A, Sells RE, Zechlinski JJ. Ewing’s sarcoma of the kidney. Int J Clin Oncol. 2010;15(3):314–18. http://dx.doi.org/10.1007/s10147-010-0042-0
20. Thyavihally YB, Tongaonkar HB, Gupta S, Kurkure PA, Amare P, Muckaden MA, et al. Primitive neuroectodermal tumor of the kidney: A single institute series of 16 patients. Urology. 2008;71(2):292–6. http://dx.doi.org/10.1016/j. urology.2007.09.051
21. Kushner BH, Hajdu SI, Gulati SC, Erlandson RA, Exelby PR, Lieberman PH. Extracranial primitive neuro-ectodermal tumors. The Memorial Sloan-Kettering Cancer Center experience. Cancer. 1991;67(7):1825–9. http://dx.doi. org/10.1002/1097-0142(19910401)67:7%3C1825::AID-CN-CR2820670702%3E3.0.CO;2-3
22. Miser JS, Kinsella TJ, Triche TJ, Steis R, Tsokos M, Wesley R, et al. Treatment of peripheral neuroepithelioma in children and young adults. J Clin Oncol. 1987;5(11):1752–8. http://dx.doi. org/10.1200/JCO.19220.127.116.112
23. Cuesta Alcalá JA, Solchaga Martínez A, Caballero Martínez MC, Gómez Dorronsoro M, Pascual Piédrola I, Ripa Saldías L, et al. Primary neuroectodermal tumour (PNET) of the kidney: 26 cases. Current status of its diagnosis and treatment. Arch Esp Urol. 2001 Dec;54(10):1081–93.