The Ewing’s Sarcoma Family of Tumors of Urinary Bladder: A Case Report and Review of the Literature

Abstract

Background: Only 15 cases of Ewing’s Sarcoma (EWS) family of tumors of urinary bladder have been documented in the literature to date. Case Report: We presented here a 38 year-old female with primary urinary bladder EWS with no distant metastases. She had presented with macroscopic hematuria and had undergone transurethral resection of the tumor within the following week. Microscopic examination revealed a tumor diffusely infiltrating the lamina propria and muscularis propria under an intact urothelium, which was composed of small round blue cells with scant cytoplasm, monotonous round or oval nuclei, stippled chromatin and small nucleoli. Immunohistochemistry showed strong vimentin, synaptophysin and membranous CD99 expression by the tumor. Fluorescent in situ hybridization analysis displayed the EWSR1 rearrangement. Radical cystectomy with total abdominal hysterectomy and bilateral salpingo-oophorectomy, extended lymph node dissection, and ileal conduit were performed. As adjuvant chemotherapy, she received vincristine, doxorubicin, cyclophosphamide and mesna, alternating with courses of etoposide, iphosphamide and mesna. She is alive and well with no evidence of disease 14 months after the surgery. Conclusion: Surgery supported with chemotherapy should be considered as an option, especially in advanced Ewing’s sarcoma family of tumors of urinary bladder.

Keywords

• Ewing’s Sarcoma, primitive neuroectodermal tumor, urinary bladder tumor

References

1. 1. Esiashvili N, Goodman M, Marcus RB Jr. Changes in incidence and survival of Ewing sarcoma patients over the past 3 decades: Surveillance Epidemiology and End Results data. J Pediatr Hematol Oncol 2008;30:425-30. [Crossref]
2. 2. Mani S, Dutta D, De BK. Primitive Neuroectodermal Tumor of the Liver: A Case Report. Jpn J Clin Oncol 2010;40:258-62. [Crossref]
3. 3. Bernstein M, Kovar H, Paulussen M, Randall RL, Schuck A, Teot LA, et al. Ewing’s sarcoma family of tumors: current management. Oncologist 2006;11:503-19. [Crossref]
4. 4. Busato WF Jr, Almeida GL, Ogata DC. Primary primitive neuroectodermal tumor of the bladder: histologic and clinical features of 9 cases. Clin Genitourin Cancer 2011;9:63-7. [Crossref]
5. 5. Osone S, Hosoi H, Tanaka K, Tsuchiya K, Iehara T, Morimoto A, et al. A case of a ewing sarcoma family tumor in the urinary bladder after treatment for acute lymphoblastic leukemia. J Pediatr Hematol Oncol 2007;29:841-4. [Crossref]
6. 6. Al Meshaan MK, Nayef M, Kwaider T, Otto W, Katchy KC. Peripheral primitive neuroectodermal tumor of the urinary bladder in an Arab woman with history of squamous cell carcinoma: a case report. J Med Case Rep 2009;3:6840. [Crossref]
7. 7. Rao RN, Sinha S, Babu S, Mehrotra R. Fine-needle aspiration cytology of primitive neuroectodermal tumor of the urinary bladder: a case report. Diagn Cytopathol 2011;39:924-6. [Crossref]
8. 8. Okada Y, Kamata S, Akashi T, Kurata M, Nakamura T, Kihara K. Primitive neuroectodermal tumor/Ewing’s sarcoma of the urinary bladder: a case report and its molecular diagnosis. Int J Clin Oncol 2011;16:435-8. [Crossref]

9. 9. Zheng Y, Tan F, Wang L, Xu N, Mou H. Primary primitive neuroectodermal tumor of the urinary bladder: a case report and literature review. Med Oncol 2011;28(Suppl 1):S388-91. [Crossref]
10. 10. Sueyoshi R, Okawada M, Fujimura J, Saito M, Koga H, Lane GJ, et al. Successful complete resection of Ewing sarcoma arising from the bladder in a 10-year-old boy after chemotherapy. Pediatr Surg Int 2014;30:965-9. [Crossref]
11. 11. Aurias A, Rimbaut C, Buffe D, Dubousset J, Mazabraud A. Chromosomal translocations in Ewing’s sarcoma. N Engl J Med 1983;309:496-7. [Crossref]
12. 12. Sorensen PH, Lessnick SL, Lopez-Terrada D, Liu XF, Triche TJ, Denny CT. A second Ewing’s sarcoma translocation, t(21;22), fuses the EWS gene to another ETS-family transcription factor, ERG. Nat Genet 1994;6:146-51. [Crossref]
13. 13. Jeon IS, Davis JN, Braun BS, Sublett JE, Roussel MF, Denny CT, et al. A variant Ewing’s sarcoma translocation (7;22) fuses the EWS gene to the ETS gene ETV1. Oncogene 1995;10:1229-34.
14. 14. Peter M, Couturier J, Pacquement H, Michon J, Thomas G, Magdelenat H, et al. A new member of the ETS family fused to EWS in Ewing tumors. Oncogene 1997;14:1159-64. [Crossref]
15. 15. Urano F, Umezawa A, Hong W, Kikuchi H, Hata J. A novel chimera gene between EWS and E1A-F, encoding the adenovirus E1A enhancer-binding protein, in extraosseous Ewing’s sarcoma. Biochem Biophys Res Commun 1996;219:608-12. [Crossref]
16. 16. Nesbit ME Jr, Gehan EA, Burgert EO Jr, Vietti TJ, Cangir A, Tefft M, et al. Multimodal therapy for the management of primary, nonmetastatic Ewing’s sarcoma of bone: a long-term follow-up of the First Intergroup study. J Clin Oncol 1990;8:1664-74.
17. 17. Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectoder