Primary Paraesophageal Ewing's Sarcoma: an Uncommon Case Report and Literature Review

Overview

Journal Onco Targets Ther

Publisher Dove Medical Press

Specialty Oncology

Date 2015 May 23

PMID 25999740

Citations 4

Authors

Noelia Tarazona

Lara Navarro

Juan Miguel Cejalvo

Valentina Gambardella

J Alejandro Perez-Fidalgo

Alejo Sempere

Samuel Navarro

Andres Cervantes

Affiliations

Soon will be listed here.

Abstract

Ewing's sarcoma is a rare and highly aggressive cancer most frequently arising in people under 20 years of age. We report an uncommon case of primary paraesophageal Ewing's sarcoma in a 25-year-old male harboring the infrequent EWSR1/ERG fusion transcript with multiple splice variants coexisting in the same tumor. The patient was totally refractory to chemotherapy and died 17 months after diagnosis. We underscore the need for better understanding of the molecular pathogenesis of the disease and improved systemic therapy options.

Citing Articles

Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Thoracic Esophagus: Case Report and Comprehensive Literature Review.

Li J, Sun P, Ma L, Min X, Ye B, Zhang Y Case Rep Oncol. 2022; 15(1):267-276.

PMID: 35529299 PMC: 9035948. DOI: 10.1159/000522152.

Extra Skeletal Ewing's Sarcoma: a Case Report.

Mir Mohmmad Sadeghi H, Mashhadi Abbas F, Taghavi N, Baharnoori N J Dent (Shiraz). 2020; 21(1):73-76.

PMID: 32158788 PMC: 7036358. DOI: 10.30476/DENTJODS.2019.44908.

Defining a Characteristic Gene Expression Set Responsible for Cancer Stem Cell-Like Features in a Sub-Population of Ewing Sarcoma Cells CADO-ES1.

Hotfilder M, Mallela N, Seggewiss J, Dirksen U, Korsching E Int J Mol Sci. 2018; 19(12).

PMID: 30563222 PMC: 6321634. DOI: 10.3390/ijms19123908.

MiR-107 suppresses cell proliferation and tube formation of Ewing sarcoma cells partly by targeting HIF-1β.

Chen J, Zhou X, Xiao Q, Wang T, Shao G, Li Y Hum Cell. 2017; 31(1):42-49.

PMID: 29075999 DOI: 10.1007/s13577-017-0183-9.

References

Patocs B, Nemeth K, Garami M, Arato G, Kovalszky I, Szendroi M . Multiple splice variants of EWSR1-ETS fusion transcripts co-existing in the Ewing sarcoma family of tumors. Cell Oncol (Dordr). 2013; 36(3):191-200. DOI: 10.1007/s13402-013-0126-8. View

Brenner J, Feng F, Han S, Patel S, Goyal S, Bou-Maroun L . PARP-1 inhibition as a targeted strategy to treat Ewing's sarcoma. Cancer Res. 2012; 72(7):1608-13. PMC: 3319786. DOI: 10.1158/0008-5472.CAN-11-3648. View

Zoubek A, Dockhorn-Dworniczak B, Delattre O, Christiansen H, Niggli F, Smith T . Does expression of different EWS chimeric transcripts define clinically distinct risk groups of Ewing tumor patients?. J Clin Oncol. 1996; 14(4):1245-51. DOI: 10.1200/JCO.1996.14.4.1245. View

Naing A, Kurzrock R, Burger A, Gupta S, Lei X, Busaidy N . Phase I trial of cixutumumab combined with temsirolimus in patients with advanced cancer. Clin Cancer Res. 2011; 17(18):6052-60. PMC: 3176947. DOI: 10.1158/1078-0432.CCR-10-2979. View

Leavey P, Mascarenhas L, Marina N, Chen Z, Krailo M, Miser J . Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multi-modality therapy: A report from the Children's Oncology Group. Pediatr Blood Cancer. 2008; 51(3):334-8. PMC: 2728357. DOI: 10.1002/pbc.21618. View

Stolow D, Haynes S . Cabeza, a Drosophila gene encoding a novel RNA binding protein, shares homology with EWS and TLS, two genes involved in human sarcoma formation. Nucleic Acids Res. 1995; 23(5):835-43. PMC: 306767. DOI: 10.1093/nar/23.5.835. View

Cabarcos Ortiz De Barron A, Cinza Sanjurjo S, Rivero Velasco C, Marino Rozados A, Antunez Lopez J . [Esophageal Ewing's sarcoma with tracheal invasion with presentation as asthmatic episode]. Rev Clin Esp. 2007; 207(6):310-1. DOI: 10.1157/13106858. View

Cope J . A viral etiology for Ewing's sarcoma. Med Hypotheses. 2000; 55(5):369-72. DOI: 10.1054/mehy.2000.1069. View

Glade Bender J, Adamson P, Reid J, Xu L, Baruchel S, Shaked Y . Phase I trial and pharmacokinetic study of bevacizumab in pediatric patients with refractory solid tumors: a Children's Oncology Group Study. J Clin Oncol. 2008; 26(3):399-405. DOI: 10.1200/JCO.2007.11.9230. View

10.

Hense H, Ahrens S, Paulussen M, Lehnert M, Jurgens H . [Descriptive epidemiology of Ewing's tumor--analysis of German patients from (EI)CESS 1980-1997]. Klin Padiatr. 1999; 211(4):271-5. DOI: 10.1055/s-2008-1043799. View

11.

Terrier-Lacombe M, Guillou L, Chibon F, Gallagher G, Benhattar J, Terrier P . Superficial primitive Ewing's sarcoma: a clinicopathologic and molecular cytogenetic analysis of 14 cases. Mod Pathol. 2008; 22(1):87-94. DOI: 10.1038/modpathol.2008.156. View

12.

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

13.

Johnson A, Pambuccian S, Andrade R, Dolan M, Aslan D . Ewing sarcoma and primitive neuroectodermal tumor of the esophagus: report of a case and review of literature. Int J Surg Pathol. 2008; 18(5):388-93. DOI: 10.1177/1066896908316903. View

14.

Granowetter L, West D . The Ewing's sarcoma family of tumors: Ewing's sarcoma and peripheral primitive neuroectodermal tumor of bone and soft tissue. Cancer Treat Res. 1997; 92:253-308. DOI: 10.1007/978-1-4615-5767-8_9. View

15.

Casey D, Wexler L, Merchant M, Chou A, Merola P, Price A . Irinotecan and temozolomide for Ewing sarcoma: the Memorial Sloan-Kettering experience. Pediatr Blood Cancer. 2009; 53(6):1029-34. DOI: 10.1002/pbc.22206. View

16.

Cantile M, Marra L, Franco R, Ascierto P, Liguori G, De Chiara A . Molecular detection and targeting of EWSR1 fusion transcripts in soft tissue tumors. Med Oncol. 2013; 30(1):412. PMC: 3586390. DOI: 10.1007/s12032-012-0412-8. View

17.

Navarro S, Cavazzana A, Llombart-Bosch A, Triche T . Comparison of Ewing's sarcoma of bone and peripheral neuroepithelioma. An immunocytochemical and ultrastructural analysis of two primitive neuroectodermal neoplasms. Arch Pathol Lab Med. 1994; 118(6):608-15. View

18.

Ferrari S, Brach Del Prever A, Palmerini E, Staals E, Berta M, Balladelli A . Response to high-dose ifosfamide in patients with advanced/recurrent Ewing sarcoma. Pediatr Blood Cancer. 2009; 52(5):581-4. DOI: 10.1002/pbc.21917. View

19.

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

20.

Siegel R, Ryan L, Antman K . Adults with Ewing's sarcoma. An analysis of 16 patients at the Dana-Farber Cancer Institute. Am J Clin Oncol. 1988; 11(6):614-7. View