title: Sclerosing epithelioid fibrosarcoma of the bone: a case report of high resistance to chemotherapy and a survey of the literature.
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.
authors.affiliation: Department of Pediatrics and Pediatric Oncology Center (POC), Klinikum rechts der Isar, Technische Universität München, Kölner Platz 1, 80804 Munich, Germany.
authors.forename: Thomas G P
authors.initials: TG
authors.lastname: Grunewald
authors.forename: Irene
authors.initials: I
authors.lastname: von Luettichau
authors.forename: Gregor
authors.initials: G
authors.lastname: Weirich
authors.forename: Angela
authors.initials: A
authors.lastname: Wawer
authors.forename: Uta
authors.initials: U
authors.lastname: Behrends
authors.forename: Peter M
authors.initials: PM
authors.lastname: Prodinger
authors.forename: Gernot
authors.initials: G
authors.lastname: Jundt
authors.forename: Stefan S
authors.initials: SS
authors.lastname: Bielack
authors.forename: Reiner
authors.initials: R
authors.lastname: Gradinger
authors.forename: Stefan
authors.initials: S
authors.lastname: Burdach
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