Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2013 Dec 18;3(1):16.
doi: 10.1186/2045-3329-3-16.

Anthracycline-based chemotherapy in extraskeletal myxoid chondrosarcoma: a retrospective study

Silvia Stacchiotti  1 , Gian Paolo Dagrada, Roberta Sanfilippo, Tiziana Negri, Isabella Vittimberga, Stefano Ferrari, Federica Grosso, Gaetano Apice, Marco Tricomi, Chiara Colombo, Alessandro Gronchi, Angelo P Dei Tos, Silvana Pilotti, Paolo G Casali
Affiliations

Anthracycline-based chemotherapy in extraskeletal myxoid chondrosarcoma: a retrospective study

Silvia Stacchiotti et al. Clin Sarcoma Res. .

Abstract

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.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Response to chemotherapy with epirubicin and ifosfamide. CT scan (arterial phase after contrast medium). Thigh primary extraskeletal myxoid chondrosarcoma (Panel A1) with concomitant intra-abdominal lymphonodes involvement (Panel A2) at baseline, and after 6 cycles of treatment with epirubicin and ifosfamide (Panel B1 and B2, respectively). The response is marked by a >30% decrease in tumor size thus classifying for a partial response by RECIST.
Figure 2
Figure 2
Overall progression free survival (PFS) of patients treated with anthracycline-based chemotherapy. Median PFS 8 months.
See this image and copyright information in PMC

References

    1. Fletcher CDM, eds. World Health Organization (WHO) Classification of Tumours of Soft tissue and Bone. Pathology and Genetics. Lyon: IARC Press; 2013.
    1. Meis-Kindblom JM, Bergh P, Gunterberg B, Kindblom LG. Extraskeletal myxoid chondrosarcoma: a reappraisal of its morphologic spectrum and prognostic factors based on 117 cases. Am J Surg Pathol. 1999;3:636–650. doi: 10.1097/00000478-199906000-00002. - DOI - PubMed
    1. Demicco EG, Wang WL, Madewell JE, Huang D, Bui MM, Bridge JA, Meis JM. Osseous myxochondroid sarcoma: a detailed study of 5 cases of extraskeletal myxoid chondrosarcoma of the bone. Am J Surg Pathol. 2013;3:752–762. doi: 10.1097/PAS.0b013e3182796e46. - DOI - PubMed
    1. Antonescu CR, Argani P, Erlandson RA. et al.Skeletal and extraskeletal myxoid chondrosarcoma: a comparative clinicopathologic, ultrastructural, and molecular study. Cancer. 1998;3:1504–1521. doi: 10.1002/(SICI)1097-0142(19981015)83:8<1504::AID-CNCR5>3.0.CO;2-B. - DOI - PubMed
    1. Hinrichs SH, Jaramillo MA, Gumerlock PH. et al.Myxoid chondrosarcoma with a translocation involving chromosomes 9 and 22. Cancer Genet Cytogenet. 1985;3:219–226. doi: 10.1016/0165-4608(85)90187-6. - DOI - PubMed