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DOI: 10.1055/s-2007-989342
© Georg Thieme Verlag KG Stuttgart · New York
Askin's Tumor: A Rare Chest Wall Neoplasm. Case Report and Short Review
Publication History
Received July 6, 2007
Publication Date:
09 July 2008 (online)
Introduction
Askin's tumor is an uncommon, malignant, small round cell tumor which belongs to the Ewing's sarcoma family of tumors (ESFT) [[1]]. More specifically, it is a peripheral primitive neuroectodermal tumor (PNET) located exclusively in the thoracopulmonary region, which was first described in childhood by Askin et al. in 1979 [[2]]. The prognosis is usually dismal with extremely low 6-year survival rates [[3]]. However, very few cases have been reported with good long-term survival rates [[1], [4]]. We present here a patient with an Askin's tumor, who was treated satisfactorily with aggressive multimodal treatment. The patient has not suffered any recurrence at the time when this report was written. This is the first, well documented case of Askin's tumor in Greece. Especial emphasis is placed on the immunohistological features and the currently established therapeutic regimen for this rare entity.
References
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- 2 Askin F B, Rosai J, Sibley R K, Dehner L P, McAllister W H. Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinico-pathologic entity of uncertain histogenesis. Cancer. 1979; 43 2438-2451
- 3 Contesso G, Llombart-Bosch A, Terrier P. et al . Does malignant small round cell tumor of the thoracopulmonary region (Askin tumor) constitute a clinicopathologic entity? An analysis of 30 cases with immunohistochemical and electron-microscopic support treated at the Institute Gustave Roussy. Cancer. 1992; 69 1012-1020
- 4 Nakajima Y, Koizumi K, Hirata T. et al . Long-term survival of Askin tumor for 10 years with 2 relapses. Ann Thorac Cardiovasc Surg. 2006; 12 137-140
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Dr. Stamatis Katsenos
Department of Pneumonology
Army General Hospital of Athens
3, Ierarhou Panaretou St.
45445 Ioannina
Greece
Phone: + 30 26 51 03 52 87
Fax: + 30 26 51 02 94 32
Email: skatsenos@yahoo.gr