Extraskeletal myxoid chondrosarcoma (EMC) is a rare subgroup within soft tissue sarcomas. Its sensitivity to chemotherapy is reported to be low.

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.

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.

By contrast to what reported so far, anthracycline-based chemotherapy is active in a distinct proportion of EMC patients.