Extraskeletal Myxoid Chondrosarcoma: State of the Art and Current Research on Biology and Clinical Management

Overview

Journal Cancers (Basel)

Publisher MDPI

Specialty Oncology

Date 2020 Sep 24

PMID 32967265

Citations 24

Authors

Silvia Stacchiotti

Giacomo Giulio Baldi

Carlo Morosi

Alessandro Gronchi

Roberta Maestro

Affiliations

Soon will be listed here.

Abstract

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 gene, which can be fused in-frame with different partners, most often or . 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.

Citing Articles

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The Role of Adenosine in Overcoming Resistance in Sarcomas.

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Clinicopathological analysis of rearranged sarcoma in the left forearm: A case report.

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UK guidelines for the management of soft tissue sarcomas.

Hayes A, Nixon I, Strauss D, Seddon B, Desai A, Benson C Br J Cancer. 2024; 132(1):11-31.

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