Abstract
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.
Introduction
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.
Discussion
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)
Conclusion
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.
Abbreviations(s)
EES: extra-osseous Ewing's sarcoma
MRI: Magnetic resonance imaging
References
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.
4. Raney RB, Asmar L Newton WA Jr, Bagwell C, Breneman JC. Crist W et al. Ewings sarcoma of soft tissues in childhood: a report from the intergroup rhabdomyosarcoma study, 1972 to 1991 . J Clin Oncol 1997; 15: 572-82.
5. Miller ME, Emerson L, Clayton F, Bentz BG, Data RE, Salzman KL, Smith LM, Yu MK. Extraosseous Ewing's sarcoma. J Clin Oncol. 2007 Oct 20;25(30):4845-8.
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.
8. O'Keeffe F, Lorigan JG, Wallace S. Radiological features of extraskelétal Ewings sarcoma. Br J Radiol 1990; 63: 456-60.
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
10. Lipski SM, Cermak K, Shumelinsky F, Gil T, Gebhart MJ. Extra-skeletal Ewing's sarcoma in adults: presentation of two cases. Acta Orthop Belg. 2010 Dec;76(6):844-9.
11. Karikari IO, Mehta AI, Nimjee S, Hodges TR, Tibaleka J, Montgomery C, Simpson L, Cummings TJ, Bagley CA. Primary intradural extraosseous Ewing sarcoma of the spine: case report and literature review. Neurosurgery. 2011 Oct;69(4):E995-9.
12. Pritchard DJ, Dahlin DC, Dauphine RT. Ewing's sarcoma. J Bone Joint Surg 1974;57:10.
13. Enzinger FM, Weiss SW. Extraskeletai Ewing's sarcoma. Soft Tissue Tumors, 2é édition, 1988:951-8.
14. Allarn K, Sze G. MR primary extraosseus Ewing sarcoma. AJNR 1994;15: 305-7.
15. Hurie J, Sariego J. Extraosseous Ewing's sarcoma. Am Surg. 2009 Dec;75(12):1255-7.
16. Zagar TM, Triche TJ, Kinsella TJ. Extraosseous Ewing's sarcoma: 25 years later. J Clin Oncol. 2008 Sep 10;26(26):4230-2.
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