Sclerosing Epithelioid Fibrosarcoma of the Bone: A Case Report of High Resistance to Chemotherapy and a Survey of the Literature

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

doi:10.1155/2010/431627

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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. Department of Pediatrics and Pediatric Oncology Center (POC), Klinikum rechts der Isar, Technische Universität München, Kölner Platz 1, 80804 Munich, Germany
von Luettichau, Irene Department of Pediatrics and Pediatric Oncology Center (POC), Klinikum rechts der Isar, Technische Universität München, Kölner Platz 1, 80804 Munich, Germany
Weirich, Gregor Institute of Pathology, Technische Universität München, Ismaninger Straße 22, 81675 Munich, Germany
Wawer, Angela Department of Pediatrics and Pediatric Oncology Center (POC), Klinikum rechts der Isar, Technische Universität München, Kölner Platz 1, 80804 Munich, Germany
Behrends, Uta Department of Pediatrics and Pediatric Oncology Center (POC), Klinikum rechts der Isar, Technische Universität München, Kölner Platz 1, 80804 Munich, Germany
Prodinger, Peter M. Department for Orthopedics and Trauma Surgery, Klinikum rechts der Isar, Technische Universität München, Ismaninger Straße 22, 81675 Munich, Germany
Jundt, Gernot Institute of Pathology, University of Basel, Schönbeinstraße 40, 4003 Basel, Switzerland
Bielack, Stefan S. Klinik für Kinder- und Jugendmedizin, Pädiatrie 5 (Onkologie, Hämatologie, Immunologie), Klinikum Stuttgart, Olgahospital, Bismarckstraße 8, 70176 Stuttgart, Germany
Gradinger, Reiner Department for Orthopedics and Trauma Surgery, Klinikum rechts der Isar, Technische Universität München, Ismaninger Straße 22, 81675 Munich, Germany
Burdach, Stefan Department of Pediatrics and Pediatric Oncology Center (POC), Klinikum rechts der Isar, Technische Universität München, Kölner Platz 1, 80804 Munich, Germany

Published in:

Sarcoma. - Hindawi Limited. - 2010, vol. 2010, p. 1-5

Oncology

Radiology Nuclear Medicine and imaging

English 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.

Language

English

Open access status

gold

Identifiers

DOI 10.1155/2010/431627
ISSN 1357-714X

Persistent URL

https://sonar.ch/global/documents/94193

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