A Primary Ewing's Sarcoma of the Kidney: A Case Report and Review of Literature

Overview

Journal Urol Ann

Specialty Urology

Date 2023 Sep 4

PMID 37664090

Authors

Ornsinee Senkhum

Worapat Attawettayanon

Choosak Pripatnanont

Virote Chalieopanyarwong

Affiliations

Soon will be listed here.

Abstract

Primary Ewing's kidney sarcoma is a rare and aggressive cancer with poor treatment outcomes. Furthermore, clinical presentations are nonspecific, such as abdominal pain, abdominal mass, cachexia, or hematuria. Currently, there is no consensus guideline for the treatment of this condition. We report on a 22-year-old man who presented with gross hematuria for 3 weeks. Computed tomography demonstrated a huge right renal mass with a thrombus in the inferior vena cava (IVC) without visceral metastasis. A percutaneous tissue biopsy revealed Ewing's sarcoma (ES) and the patient received neoadjuvant chemotherapy (doxorubicin and ifosfamide) for four cycles. This reduced the tumor's size significantly and made it eligible for surgical intervention. Radical nephrectomy and tumor thrombus removal with wedge IVC wall were performed. The final diagnosis of ES was confirmed by the immunohistochemistry of the kidney specimen. After a 5-year follow-up of the patient, clinical and imaging evidence demonstrated that there was no disease recurrence.

References

Zhang S, Li Y, Wang R, Song B . Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a case report and literature review. Transl Androl Urol. 2019; 8(5):562-566. PMC: 6842790. DOI: 10.21037/tau.2019.09.46. View

Cheng L, Xu Y, Song H, Huang H, Zhuo D . A rare entity of Primary Ewing sarcoma in kidney. BMC Surg. 2020; 20(1):280. PMC: 7661154. DOI: 10.1186/s12893-020-00948-9. View

Celli R, Cai G . Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: A Rare and Lethal Entity. Arch Pathol Lab Med. 2016; 140(3):281-5. DOI: 10.5858/arpa.2014-0367-RS. View

Bontoux C, Khaddour S, Perot G, Vaessen C, Boostandoost H, Augustin J . Case report of an ewing's sarcoma/primitive neuroectodermal tumor of the kidney. Int Urol Nephrol. 2018; 50(8):1449-1451. DOI: 10.1007/s11255-018-1922-x. View

Grier H, Krailo M, Tarbell N, Link M, Fryer C, Pritchard D . Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. 2003; 348(8):694-701. DOI: 10.1056/NEJMoa020890. View

Zollner S, Dirksen U, Jurgens H, Ranft A . Renal Ewing tumors. Ann Oncol. 2013; 24(9):2455-61. DOI: 10.1093/annonc/mdt215. View

Teegavarapu P, Rao P, Matrana M, Cauley D, Wood C, Patel S . Outcomes of Adults With Ewing Sarcoma Family of Tumors (ESFT) of the Kidney: A Single-Institution Experience. Am J Clin Oncol. 2014; 40(2):189-193. PMC: 4372498. DOI: 10.1097/COC.0000000000000128. View

Fizazi K, Dohollou N, Blay J, Guerin S, Le Cesne A, Andre F . Ewing's family of tumors in adults: multivariate analysis of survival and long-term results of multimodality therapy in 182 patients. J Clin Oncol. 1998; 16(12):3736-43. DOI: 10.1200/JCO.1998.16.12.3736. View

El Weshi A, Allam A, Ajarim D, Al Dayel F, Pant R, Bazarbashi S . Extraskeletal Ewing's sarcoma family of tumours in adults: analysis of 57 patients from a single institution. Clin Oncol (R Coll Radiol). 2010; 22(5):374-81. DOI: 10.1016/j.clon.2010.02.010. View

10.

Doroudinia A, Ahmadi S, Mehrian P, Pourabdollah M . Primary Ewing sarcoma of the kidney. BMJ Case Rep. 2019; 12(1). PMC: 6350699. DOI: 10.1136/bcr-2018-227198. View

11.

Dahlin D, COVENTRY M, SCANLON P . Ewing's sarcoma. A critical analysis of 165 cases. J Bone Joint Surg Am. 1961; 43-A:185-92. View

12.

Rowe R, Thomas D, Schuetze S, Hafez K, Lawlor E, Chugh R . Ewing sarcoma of the kidney: case series and literature review of an often overlooked entity in the diagnosis of primary renal tumors. Urology. 2013; 81(2):347-53. DOI: 10.1016/j.urology.2012.10.016. View

13.

Richey S, Rao P, Wood C, Patel S, Tannir N . Metastatic extraosseous Ewing's sarcoma (EES)/primitive neuroectodermal tumor (PNET) of the kidney: 8-year durable response after induction and maintenance chemotherapy. Clin Genitourin Cancer. 2012; 10(3):210-2. PMC: 4121061. DOI: 10.1016/j.clgc.2012.03.004. View

14.

Seemayer T, Thelmo W, BOLANDE R, WIGLESWORTH F . Peripheral neuroectodermal tumors. Perspect Pediatr Pathol. 1975; 2:151-72. View

15.

Llombart-Bosch A, Machado I, Navarro S, Bertoni F, Bacchini P, Alberghini M . Histological heterogeneity of Ewing's sarcoma/PNET: an immunohistochemical analysis of 415 genetically confirmed cases with clinical support. Virchows Arch. 2009; 455(5):397-411. DOI: 10.1007/s00428-009-0842-7. View

16.

Findlay B, Shinder B, Fatima A, Sadimin E, Drachtman R, Parikh R . Primary Renal Ewing Sarcoma: A Case Report and Review of the Literature. J Ren Cancer. 2020; 2(1):15-19. PMC: 7034944. View