Case Report

By Dr. Najib Alidrissi
Corresponding Author Dr. Najib Alidrissi
University Mohammed V - Souissi , - Morocco 10000
Submitting Author Dr. Najib Alidrissi

Ewing's Sarcoma, Chemotherapy, Surgery

Alidrissi N. Extra Osseous Ewing's Sarcoma: A Case Report. WebmedCentral ONCOSURGERY 2012;3(6):WMC003508
doi: 10.9754/journal.wmc.2012.003508

This is an open-access article distributed under the terms of the Creative Commons Attribution License(CC-BY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Submitted on: 22 Jun 2012 06:22:05 PM GMT
Published on: 23 Jun 2012 07:27:22 PM GMT


We report a case of extraosseous Ewing's sarcoma localized in the gluteal region. The treatment consisted of chemotherapy to reduce tumor size followed by surgery.


The extra-osseous Ewing's sarcoma (EES)  is a rare tumor, which is derived from neural crest cells and integrates within the peripheral neuroectodermal tumors. (1,2) These tumors have common cytogenetic features. (2, 3)

Case Report(s)

Mr. A., aged 24 has a swelling on the right buttock, painless at rest, pain with walking. The examination was unremarkable. Plain radiographs of the hip and pelvis revealed no abnormalities in the bone (Fig. 1). The examination of the pelvis CT scan can visualize a hypodense expansive process, developed in the right gluteal muscles, without osteolysis next (fig2). The assessment of extension is normal. The anatomopathological study performed after surgical biopsy of the mass confirmed the diagnosis of extra-osseous Ewing's sarcoma. (Fig. 3) Chemotherapy was administered before the surgical treatments in the patient then underwent surgery two months later after discontinuation of chemotherapy and has received wide resection (Fig. 4), the sciatic nerve was independent of tumor mass. The immediate evolution was marked by a postoperative infection consequent to immunosuppression of the patient was under chemotherapy and has been controlled by appropriate antibiotic therapy and local care.


Unlike Ewing intraosseous, which is common in males, no predisposition in terms of sex is present. Two thirds of tumors diagnosed occur in young adults. On the etiopathogenic, ql2 translocation of chromosome 22 is found in 95-100% of cases. (4, 5, 6) Clinical signs are nonspecific. The topography is ubiquitous but preferentially reached the paravertebral region, the thoracic wall, retroperitoneum and lower extremities rather than higher. (7, 8, 9, 10) Radiologically: nonspecific mass. Most lesions are encapsulated, hypoechoic on ultrasound, hypodense on CT, it is often hyper-vascularized, so hyperdense after injection of contrast material. In MRI, the signal intensity of the tumor is iso-hypointense on T1, hyperintense on T2. (3, 11, 12) The diagnosis is the cytogenetics and immunohistochemistry, particularly through in situ hybridization by immunofluorescence. The treatment combines surgery, chemotherapy and radiotherapy. (14, 15) The Ewing's sarcoma have developed rapidly, neoadjuvant treatment (radiotherapy and chemotherapy) is made ??of this very useful in reducing tumor size and allow complete removal. The 10-year survival is possible, it would be at around 62 to 77%. The intensification of chemotherapy more aggressive may explain this difference. The term remission is possible. (16)


The EES is a rare, interesting young adults, the diagnosis is difficult even on the histology. It is very close neuroectodermal tumors in terms of ultrastructure, cytogenetics, biochemistry and immunology. In the absence of specific radiological signs, it seems necessary to include in the differential diagnosis of any primary tumor of soft parts Imaging, MRI, in particular, allows an assessment of the lesions and monitor therapy. Ewing sarcoma bone deserves extra-early diagnosis, which can offer the best chance of survival.


EES: extra-osseous Ewing's sarcoma
MRI: Magnetic resonance imaging 


1. Angervall C Enzinger FM. Extraskeletal neoplasm ressembling Ewing's sarcoma. Cancer 1975; 36: 240-51.
2. Rud NP, Reihman HM, Pritchard DJ, Frassica FJ, Smithon WA. Extraosseous Ewings sarcoma. A study of 42 cases. Cancer 1989: 64: 1548-53.
3. Fernández del Castillo Ascanio M, Sirvent Cerdá S. [Extraosseous Ewing's sarcoma]. Radiologia. 2010 May-Jun;52(3):276-7.
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6. Stuart HR, Willis EJ. Langlands AO, Fox RM, Tattersall MNH. Extraskeletal Ewing’s sarcoma: a clinical, morphological and ultrastructural analysis of five cases with a review of the litterature. Eur J Cancer Clin Oncol 1986; 22: 393-400.
7. Shimada H, Newton WA. Soule EH, Qualman SJ. Aoyama C. Maurer HM. Pathologic features of extra osseous Ewing’s sarcoma: a report from the intergroup rhabdomyosarcoma study. Hum Pathol 1988; 19; 442-53.
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9. Paoletti H,  Colineau  X.  Acalet  L , Tourrette JH , Civatte M ,  Fesselet J, Dussaut JP and al - Sarcome d'Ewing des parties molles:a propos de 3 cas et revue de la littérature. J Radial 1999;80:477-82
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