TY - JOUR
T1 - Extraskeletal myxoid chondrosarcoma
T2 - State of the art and current research on biology and clinical management
AU - Stacchiotti, Silvia
AU - Baldi, Giacomo Giulio
AU - Morosi, Carlo
AU - Gronchi, Alessandro
AU - Maestro, Roberta
N1 - Funding Information:
Conflicts of Interest: S.S. outside submitted work: advisory from Bayer, Bavarian Nordic, Deciphera, Daiichi, Eli Lilly, Epizyme, Karyopharm, MaxiVax, Pharmamar; honoraria from Eli Lilly, Pharmamar; travel grants from Pharmamar; institutional research funding from Advenchen, Amgen Dompé, AROG, Bayer, Blueprint Medicines, Daiichi Sankyo Pharma, Deciphera, Eli Lilly, Epizyme, GSK, Karyopharm, Novartis, Pfizer, PharmaMar, SpringWorks. G.G.B. outside submitted work: honoraria for consultancy from Eli Lilly, Eisai and PharmaMar; travel grants from PharmaMar, Pfizer and Eli Lilly; advisory board from AboutEvents, EditaMed, Eli Lilly. C.M., A.G. and R.M. declare no conflicts of interest.
Publisher Copyright:
© 2020, MDPI AG. All rights reserved.
Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 2020/9
Y1 - 2020/9
N2 - Extraskeletal myxoid chondrosarcoma (EMC) is an ultra-rare mesenchymal neoplasm with uncertain differentiation, which arises mostly in the deep soft tissue of proximal extremities and limb girdles. EMC is marked by a translocation involving the NR4A3 gene, which can be fused in-frame with different partners, most often EWSR1 or TAF1. Although EMC biology is still poorly defined, recent studies have started shedding light on the specific contribution of NR4A3 chimeric proteins to EMC pathogenesis and clinical outcome. Standard treatment for localized disease is surgery, plus or minus radiation therapy with an expected prolonged survival even though the risk of relapse is about 50%. In advanced cases, besides the standard chemotherapy currently used for soft tissue sarcoma, antiangiogenic agents have recently shown promising activity. The aim of this review is to provide the state of the art of treatment for localized and advanced disease, with a focus on pharmacological treatments available for EMC. The biological basis of current research and future perspectives will be also discussed.
AB - Extraskeletal myxoid chondrosarcoma (EMC) is an ultra-rare mesenchymal neoplasm with uncertain differentiation, which arises mostly in the deep soft tissue of proximal extremities and limb girdles. EMC is marked by a translocation involving the NR4A3 gene, which can be fused in-frame with different partners, most often EWSR1 or TAF1. Although EMC biology is still poorly defined, recent studies have started shedding light on the specific contribution of NR4A3 chimeric proteins to EMC pathogenesis and clinical outcome. Standard treatment for localized disease is surgery, plus or minus radiation therapy with an expected prolonged survival even though the risk of relapse is about 50%. In advanced cases, besides the standard chemotherapy currently used for soft tissue sarcoma, antiangiogenic agents have recently shown promising activity. The aim of this review is to provide the state of the art of treatment for localized and advanced disease, with a focus on pharmacological treatments available for EMC. The biological basis of current research and future perspectives will be also discussed.
KW - Antiangiogenics
KW - Chemotherapy
KW - Chondrosarcoma
KW - NR4A3 fusions
KW - Sarcoma
UR - http://www.scopus.com/inward/record.url?scp=85091679359&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85091679359&partnerID=8YFLogxK
U2 - 10.3390/cancers12092703
DO - 10.3390/cancers12092703
M3 - Review article
VL - 12
SP - 1
EP - 13
JO - Cancers
JF - Cancers
SN - 2072-6694
IS - 9
M1 - 2703
ER -