BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare subgroup within soft tissue sarcomas. Its sensitivity to chemotherapy is reported to be low. METHODS: We retrospectively reviewed a series of 11 EMC patients treated as from 2001 within the Italian Rare Cancer Network (RCN) with anthracycline-based chemotherapy. Pathologic diagnosis was centrally reviewed in all cases and confirmed by the presence of the specific chromosomal rearrangements, involving the NR4A3 gene locus on chromosome 9. RESULTS: Eleven patients treated with anthracycline-based chemotherapy were included (M/F: 9/2 - mean age: 52 years - site of primary: lower limb/other = 9/2 - metastatic = 11 - front line/ further line = 10/1 - anthracycline as single agent/ combined with ifosfamide = 1/10). Ten patients are evaluable for response. Overall, best response according to RECIST was: partial response (PR) = 4 (40 %), stable disease (SD) = 3, progressive disease (PD) = 3 cases. Median PFS was 8 (range 2-10) months. CONCLUSIONS: By contrast to what reported so far, anthracycline-based chemotherapy is active in a distinct proportion of EMC patients.
Anthracycline-based chemotherapy in extraskeletal myxoid chondrosarcoma: a retrospective study / S. Stacchiotti, G.P. Dagrada, R. Sanfilippo, T. Negri, I. Vittimberga, S. Ferrari, F. Grosso, G. Apice, M. Tricomi, C. Colombo, A. Gronchi, A.P. Dei Tos, S. Pilotti, P.G. Casali. - In: CLINICAL SARCOMA RESEARCH. - ISSN 2045-3329. - 3:1(2013 Dec 18), pp. 16.1-16.7.
|Titolo:||Anthracycline-based chemotherapy in extraskeletal myxoid chondrosarcoma: a retrospective study|
|Parole Chiave:||Sarcoma; Chondrosarcoma; Extraskeletal myxoid chondrosarcoma; Chemotherapy; Anthracycline; Doxorubicin; Ifosfamide|
|Settore Scientifico Disciplinare:||Settore MED/06 - Oncologia Medica|
|Data di pubblicazione:||18-dic-2013|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1186/2045-3329-3-16|
|Appare nelle tipologie:||01 - Articolo su periodico|