Cirugía y Cirujanos

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>Journals >Cirugía y Cirujanos >Year 2010, Issue 2

Valdivia-Gómez GG, Soto-Guerrero MT, Cedillo-de la Cruz MI
Sarcoma de Ewing extraesquelético que semeja abdomen agudo
Cir Cir 2010; 78 (2)

Language: Español
References: 31
Page: 163-166
PDF: 230.94 Kb.

[Full text - PDF]


Background: Extra-osseous Ewing’s sarcoma is a rare tumor of neuroectodermal origin. It presents mainly in the soft tissue of the extremities and thorax. Histologically, it is similar to Ewing’s sarcoma of the bone.
Clinical case: We present the case of a male who arrived at the emergency room with acute abdomen, leucocytosis and imaging techniques (abdominal ultrasound and computed tomography) suggestive of complicated diverticular disease. He was treated with emergency surgery. Intraoperative findings were an unsuspected tumor (20 × 15 × 15 cm). Treatment consisted of extirpation of the tumor, separating it from the adjacent viscera and followed by chemotherapy based on epirubicin, cyclophosphamide and vincristine for six cycles. Because the control abdominal CT demonstrated tumor activity in the retroperitoneum adjacent to the ascending colon and cecum, further resection was decided upon.
Conclusions: In a review of the literature, no previous reports of extra-osseous Ewing’s sarcoma were found presenting as acute abdomen. Due to the rarity of this tumor, only case reports or series have been found in the literature without randomized or comparative studies. Surgery was the cornerstone of treatment, without reports of preoperative chemotherapy. If the patient’s condition permits, percutaneous needle biopsy is mandatory to obtain optimum treatment as well as to improve the prognosis.

Key words: Ewing’s sarcoma, soft tissue tumor, primitive neuroectodermal tumor, neuroepithelial tumor, abdominaltumor, acute abdomen.


  1. Navarro S, Cavazanna AO, Llombart-Bosch A, Triche TJ. 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;118:608-615.

  2. Delattre O, Zucman J, Melot T, Garau XS, Zucker JM, Lenoir GM, et al. The Ewing family of tumors. A subgroup of small roundcell tumors defined by specific chimeric transcripts. N Engl J Med 1994;33:294-299.

  3. Ahmad R, Mayol BR, Davis M, Rougraff BT. Extraskeletal Ewing’s sarcoma. Cancer 1999;85:725-731.

  4. Minniti A, Jougon J, Barone M, Belleannee G, Velly JF. A case of extraskeletal Ewing’s sarcoma of the thoracic wall. G Chir 2000;21:450-452.

  5. Dickinson J, Watts AC, Robb JE. Extra-osseous Ewing’s sarcoma. J Bone Joint Surg 2009;91-B(suppl II);215.

  6. Amatruda JF, Tran K, Mantel M, Singer S, Demetri G. Diagnosis in oncology. Askin tumor. J Clin Oncol 1998;16:1997-1998.

  7. Jiménez RE, Folpe AL, Lapham RL, Ro JY, O’Shea PA, Weiss SW, et al. Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases. Am J Surg Pathol 2002;26:320-327.

  8. Ho OP, Manikandan R, Philp J, Hope K, Williamson M. Primitive neuroectodermal tumour of the kidney with vena caval and artrial tumour thrombus: a case report. J Urol 2000;164:772.

  9. Gousse AE, Roth DR, Popek EJ, Cooley LD, Horowitz ME. Primary Ewing’s sarcoma of the bladder associated with an elevated antinuclear antibody titer. J Urol 1997;158:2265-2266.

  10. Toh KL, Tan PH, Cheng WS. Primary extraskeletal Ewing’s sarcoma of the external genitalia. J Urol 1999;162:159-160.

  11. Adair A, Harris SA, Coppen MJ, Hurley PR. Extraskeletal Ewing’s sarcoma of the small bowel: case report and literature review. J R Coll Surg Edinb 2001;46:372-374.

  12. Colovic RB, Grubor NM, Micev MT, Matic SV, Atkinson HD, Latincic SM. Perigastric extraskeletal Ewing’s sarcoma: a case report. World J Gastroenterol 2009;15:245-247.

  13. Agir H, Brasch HD, Tan ST. Extra-skeletal Ewing’s sarcoma of the submandibular gland. J Plast Reconstr Aesthet Surg 2007;60:1345-1348.

  14. Yang YS, Hong KH. Extraskeletal Ewing’s sarcoma of the larynx. J Laryngol Otol 2004;118:62-64.

  15. Kourda M, Chatti S, Sfia M, Kraiem W, Ben Brahim E. Primary cutaneous extraskeletal Ewing’s sarcoma. Ann Dermatol Venereol 2005;132:986-989.

  16. Hasegawa SL, Davison JM, Rutten A, Fletcher JA, Fletcher CD. Primary cutaneous Ewing’s sarcoma: immunophenotypic and molecular cytogenetic evaluation of five cases. Am J Surg Pathol 1998;22:310-318.

  17. Li T, Goldberg RA, Becker B, Mc Cann J. Primary orbital extraskeletal Ewing sarcoma. Arch Ophthalmol 2003;121:1049-1052.

  18. Askri A, Farhat LB, Ghariani B, Rabeh A, Dali N, Said W, et al. Extraskeletal Ewing sarcoma of the abdominal wall. Cancer Imaging 2008;8:156-158.

  19. Aydinli B, Ozturk G, Yildirgan MI, Polat KY, Basoglu M, Gundogdu C, et al. Extraskeletal Ewing’s sarcoma in the abdominal wall: a case report. Acta Oncol 2006;45:404-406.

  20. Collini P, Sampietro G, Bertulli R, Casali PG, Luksch R, Mezzelani A, et al. Cytokeratin immunoreactivity in 41 cases of ES/PNET confirmed by molecular diagnostic studies. Am J Surg Pathol 2001;25:273-274.

  21. Goldblum JR, Machen SK, Fisher C. Cytokeratins in round cell sarcomas. Am J Surg Pathol 2000;24:1174-1175.

  22. de Alava E, Gerald WL. Molecular biology of the Ewing’s sarcoma/primitive neuroectodermal tumor family. J Clin Oncol 2000;18:204-213.

  23. Vogel H, Fuller GN. Primitive neuroectodermal tumors, embryonal tumors, and other small cell and poorly differentiated malignant neoplasms of the central and peripheral nervous systems. Ann Diagn Pathol 2003;7:387-398.

  24. Rosa F, Tortorelli AP, Papa U, Pacelli F, Doglietto GB. Image of the month—quiz case. Retroperitoneal extraskeletal Ewing sarcoma. Arch Surg 2008;143:511-512.

  25. Monforte-Muñoz H, López-Terrada D, Affendie H, Rowland JM, Triche TJ. Documentation of EWS gene rearrangements by fluorescence in-situ hybridization (FISH) in frozen sections of Ewing’s sarcoma-peripheral primitive neuroectodermal tumor. Am J Surg Pathol 1999;23:309-315.

  26. Gu M, Antonescu CR, Guiter G, Huvos AG, Ladanyi M, Zakowski MF. Cytokeratin immunoreactivity in Ewing's sarcoma: prevalence in 50 cases confirmed by molecular diagnostic studies. Am J Surg Pathol 2000;24:1174-1175.

  27. Guiter GE, Gamboni MM, Zakowski MF. The cytology of extraskeletal Ewing sarcoma. Cancer 1999;87:141-148.

  28. Matsuda S, Tanaka K, Chuman H, Iwamoto Y. Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. Spine 2003;28:E408-412.

  29. Tracker MM, Temple HT, Scully SP. Current treatment for Ewing’s sarcoma. Expert Rev Anticancer Ther 2005;5:319-331.

  30. Oberlin O, Le Deley MC, Bui BN, Gentet JC, Philip T, Terrier P, et al. Prognostic factors in localized Ewing’s tumours and peripheral neuroectodermal tumours: the third study of the French Society of Paediatric Oncology (EW88 study). Br J Cancer 2001;85:1646-1654.

  31. Venkitaraman R, George MK, Ramanan SG, Sagar TG. A singular institution experience of combined modality management of extra skeletal Ewing’s sarcoma. World J Surg Oncol 2007;11:5-13.

>Journals >Cirugía y Cirujanos >Year 2010, Issue 2

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