Cancer Res Treat.  2009 Jun;41(2):108-112.

Multimodal Treatment of Primary Extraskeletal Ewing's Sarcoma of the Chest Wall: Report of 2 Cases

• Lee WS
• Kim YH
• Chee HK
• Hwang JJ
• Kim JS
• Lee SA
• Hwang EG
• Cho YH
• Chon GR

Abstract

Extraskeletal Ewing's sarcoma (EES) is a type of Ewing's sarcoma that arises in soft tissue and is now regarded as a member of a family of small round cell neoplasms of bone and soft tissue, including primitive neuroectodermal tumors (PNETs). EES occurs predominantly in adolescents and young adults between the ages of 10 and 30 years. The disease follows an aggressive course with a high recurrence rate. The presence of a distant metastasis is also common. EES arises in the soft tissue of either the trunk or extremities. We recently experienced two cases of EES that occurred in the chest wall. The two patients underwent wide resection and combined radiochemotherapy. There was no evidence of disease 30 and 22 months, respectively, after surgery. Although extremely rare, EES should be considered in the differential diagnosis of chest wall tumors. We report two cases of EES with a brief review of the literature.

Keyword

Extraskeletal Ewing's sarcoma; Primitive neuroectodermal tumor

MeSH Terms

Adolescent
Chemoradiotherapy
Combined Modality Therapy
Diagnosis, Differential
Extremities
Humans
Neoplasm Metastasis
Neuroectodermal Tumors, Primitive
Recurrence
Sarcoma, Ewing
Thoracic Wall
Thorax
Young Adult

Figure

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  Fig. 1 (A) Case 1. An initial chest CT scan indicates the presence of a round mass-like lesion in the right anterior lower thorax (white arrow). (B) Case 2. An initial chest CT scan indicates the presence of a round mass-like lesion in the left lower thorax protruding to the thoracic cavity (black arrow) and an inserted chest tube (black arrowhead). (C) Case 2. A follow-up CT scan obtained six months after surgery shows no evidence of recurrence and the chest wall defect repaired with a 2-mm Gore-Tex patch.

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  Fig. 2 Operative findings are presented. (A) Case 1. The resected sixth and seventh ribs surrounded by the tumor are shown. (B) Case 1. The chest wall was repaired with a 2-mm Gore-Tex patch.

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  Fig. 3 Features of extraskeletal Ewing's sarcoma are presented. (A) Diffuse proliferation of round cells with clear cytoplasm is shown (H & E staining, ×200). (B) Diffuse proliferation of small round cells and thick fibrous septation (H & E staining, ×40) is shown. The upper box shows angioinvasion, and the lower box shows the nuclear details (H & E staining, ×100 and ×400, respectively). (C) PAS staining (×100) shows fine cytoplamic glycogen droplets. Positive immunohistochemical staining (×100) results for the use of antibodies directed against CD17, CD56 and CD99.

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