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Sclerosing epithelioid fibrosarcoma of the bone : a case report of high resistance to chemotherapy and a survey of the literature

Grunewald, Thomas G. P. and von Luettichau, Irene and Weirich, Gregor and Wawer, Angela and Behrends, Uta and Prodinger, Peter M. and Jundt, Gernot and Bielack, Stefan S. and Gradinger, Reiner and Burdach, Stefan. (2010) Sclerosing epithelioid fibrosarcoma of the bone : a case report of high resistance to chemotherapy and a survey of the literature. Sarcoma, Vol. 2010 , 431627.

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Official URL: http://edoc.unibas.ch/dok/A6006438

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Abstract

Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue sarcoma mostly occurring in extraosseous sites. SEF represents a clinically challenging entity especially because no standardized treatment regimens are available. Intraosseous localization is an additional challenge with respect to the therapeutical approach. We report on a 16-year-old patient with SEF of the right proximal tibia. The patient underwent standardized neoadjuvant chemotherapy analogous to the EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprosthetic reconstruction. Histopathological analysis of the resected tumor showed <90% vital tumor cells suggesting no response to chemotherapy. Therefore, therapy was reassigned to the CWS 2002 High-Risk protocol for the treatment of soft tissue sarcoma. To date (22 months after diagnosis), there is no evidence of relapse or metastasis. Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin, and Methotrexate, which should be considered in planning treatment for patients with SEF.
Faculties and Departments:03 Faculty of Medicine > Bereich Querschnittsfächer (Klinik) > Pathologie USB
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Querschnittsfächer (Klinik) > Pathologie USB
UniBasel Contributors:Jundt, Gernot
Item Type:Article, refereed
Article Subtype:Research Article
Bibsysno:Link to catalogue
Publisher:Hindawi
ISSN:1357-714X
Note:Publication type according to Uni Basel Research Database: Journal article
Identification Number:
Last Modified:24 May 2013 09:22
Deposited On:24 May 2013 09:06

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