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

Irene Von Luettichau, Thomas G.P. Grunewald, Gregor Weirich, Angela Wawer, Uta Behrends, Peter M. Prodinger, Gernot Jundt, Stefan S. Bielack, Reiner Gradinger, Stefan Burdach

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29 Scopus citations

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.

Original languageEnglish
Article number431627
JournalSarcoma
Volume2010
DOIs
StatePublished - 2010
Externally publishedYes

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