Extraskeletal Myxoid Chondrosarcoma

A rare malignant soft tissue neoplasm that originates from CHONDROBLASTS. It is most common in male middle-aged and elderly individuals and often presents as deep lesions in soft tissue of the extremities. Malignancy is low, but recurrence and METASTASIS can occur many years after the initial diagnosis. Histologically, the tumors consist of a mixture of cellular and myxoid stromal components. Can be caused by somatic chromosomal translocations resulting in fusions between the NR4A3 and EWSR1 genes, and NR4A3 and TAF15 genes. OMIM: 612237

Also Known As:

Networked: 70 relevant articles (2 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Chondrosarcoma
3.	Chordoma
4.	Hypoxia (Hypoxemia)
5.	Mesenchymal Chondrosarcoma

Experts

1.	Gronchi, Alessandro: 5 articles (12/2020 - 10/2012)
2.	Labelle, Yves: 5 articles (10/2012 - 09/2002)
3.	Casali, Paolo G: 4 articles (06/2014 - 10/2012)
4.	Filion, Christine: 4 articles (10/2012 - 09/2002)
5.	Dagrada, Gian Paolo: 3 articles (01/2019 - 10/2012)
6.	Stacchiotti, Silvia: 3 articles (01/2019 - 10/2012)
7.	Chow, Warren A: 2 articles (01/2022 - 07/2007)
8.	Milhem, Mohammed: 2 articles (08/2021 - 01/2020)
9.	Chezal, Jean-Michel: 2 articles (01/2021 - 01/2020)
10.	Maubert, Elise: 2 articles (01/2021 - 01/2020)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Extraskeletal Myxoid Chondrosarcoma:

1.	apatinibIBA 01/01/2020 - "Based on clinicopathological factors Cox and univariate analysis, only extraskeletal myxoid chondrosarcoma and baseline target lesions <60 mm benefited from the drug apatinib (P=0.14 and P=0.00), respectively. "
2.	Proteins (Proteins, Gene)FDA Link 10/01/2012 - "Identification of genes regulated by the EWS/NR4A3 fusion protein in extraskeletal myxoid chondrosarcoma." 12/15/2000 - "Fusion of the NH2-terminal domain of the basic helix-loop-helix protein TCF12 to TEC in extraskeletal myxoid chondrosarcoma with translocation t(9;15)(q22;q21)." 01/01/1992 - "Cellular variant of extraskeletal myxoid chondrosarcoma of abdominal wall--a case report with comparative immunohistochemical study on cartilaginous collagenous proteins in various myxoid mesenchymal tumors." 10/11/2012 - " Extraskeletal myxoid chondrosarcoma (EMCS) is a rare soft tissue sarcoma of uncertain differentiation, characterized in most cases by a translocation that results in the fusion protein EWSR1-CHN (the latter even called NR4A3 or TEC). " 10/01/2012 - "Approximately 75 % of extraskeletal myxoid chondrosarcoma tumors (EMC) harbor a t(9;22) chromosome translocation generating an EWS/NR4A3 fusion protein that is thought to be instrumental in the tumoral process. "
3.	Orphan Nuclear ReceptorsIBA 07/01/2007 - "Molecular studies have established that extraskeletal myxoid chondrosarcoma is a unique entity defined by the presence of a fusion gene between the orphan nuclear receptor, CHN/NOR1, and a promiscuous partner, most commonly EWSR1. " 03/01/1997 - "Molecular analysis of the fusion of EWS to an orphan nuclear receptor gene in extraskeletal myxoid chondrosarcoma." 10/15/2008 - "In extraskeletal myxoid chondrosarcoma, a chromosomal translocation creates a gene fusion between EWS and an orphan nuclear receptor, NOR1. " 01/04/2002 - "In extraskeletal myxoid chondrosarcoma, chromosomal translocation creates a gene fusion between EWS and the orphan nuclear receptor NOR1. "
4.	ProdrugsIBA 01/01/2021 - "Relevance of the combination of external beam radiotherapy with the hypoxia-activated prodrug ICF05016 in an experimental model of extraskeletal myxoid chondrosarcoma." 01/01/2020 - "In a previous work, ICF05016 was identified as efficient proteoglycan-targeting hypoxia-activated prodrug in a human extraskeletal myxoid chondrosarcoma model in mice and a first study of the structure-activity relationship of the QA function and the alkyl linker length was conducted. "
5.	AntibodiesIBA 07/01/2009 - "Supraclavicular extraskeletal myxoid chondrosarcoma presenting with a sensorimotor polyneuropathy associated with anti-Hu antibodies." 05/01/2009 - "In this study, immunohistochemical staining was performed with antibodies against D2-40, S100, pankeratin, epithelial membrane antigen (EMA), brachyury, and glial fibrillary acidic protein (GFAP) in 4 cases of chordoid glioma, 6 skeletal myxoid chondrosarcomas, 10 chordoid meningiomas, 16 extraskeletal myxoid chondrosarcoma, 18 chordomas, 22 low-grade chondrosarcomas, and 27 enchondromas. "
6.	pazopanibFDA Link 01/01/2019 - "Pazopanib for treatment of advanced extraskeletal myxoid chondrosarcoma: a multicentre, single-arm, phase 2 trial." 01/01/2019 - "We aimed to investigate the activity of pazopanib, an antiangiogenic drug, in patients with advanced extraskeletal myxoid chondrosarcoma. " 01/01/2019 - "Pazopanib had clinically meaningful antitumour activity in patients with progressive and advanced extraskeletal myxoid chondrosarcoma, and could be considered a suitable option after failure to respond to first-line anthracycline-based chemotherapy in these patients. " 06/01/2014 - "Recent discoveries about molecular pathways involved in STS tumorogenesis led to develop molecular targeted agents such as imatinib used in advanced dermatofibrosarcoma protuberans (DFSP) or metastatic DFSP-related fibrosarcoma, pazopanib, approved as second line regimen in advanced non-adipocitic STS and recently sunitinib in solitary fibrous tumors, alveolar soft part sarcoma and extraskeletal myxoid chondrosarcoma."
7.	ICF05016IBA 01/01/2021 - "Relevance of the combination of external beam radiotherapy with the hypoxia-activated prodrug ICF05016 in an experimental model of extraskeletal myxoid chondrosarcoma." 01/01/2020 - "In a previous work, ICF05016 was identified as efficient proteoglycan-targeting hypoxia-activated prodrug in a human extraskeletal myxoid chondrosarcoma model in mice and a first study of the structure-activity relationship of the QA function and the alkyl linker length was conducted. "
8.	Mucin-1 (CA 15-3 Antigen)IBA 05/01/2009 - "In this study, immunohistochemical staining was performed with antibodies against D2-40, S100, pankeratin, epithelial membrane antigen (EMA), brachyury, and glial fibrillary acidic protein (GFAP) in 4 cases of chordoid glioma, 6 skeletal myxoid chondrosarcomas, 10 chordoid meningiomas, 16 extraskeletal myxoid chondrosarcoma, 18 chordomas, 22 low-grade chondrosarcomas, and 27 enchondromas. "
9.	Complementary DNA (cDNA)IBA 03/01/2003 - "Studies on the molecular pathogenesis of extraskeletal myxoid chondrosarcoma-cytogenetic, molecular genetic, and cDNA microarray analyses."
10.	Proteoglycans (Proteoglycan)IBA 01/01/2020 - "In a previous work, ICF05016 was identified as efficient proteoglycan-targeting hypoxia-activated prodrug in a human extraskeletal myxoid chondrosarcoma model in mice and a first study of the structure-activity relationship of the QA function and the alkyl linker length was conducted. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/2010 - "Extraskeletal myxoid chondrosarcoma: a case report of complete remission by chemotherapy and review of the literature." 12/15/2008 - "Extraskeletal myxoid chondrosarcoma: a retrospective review from 2 referral centers emphasizing long-term outcomes with surgery and chemotherapy." 01/01/2019 - "Extraskeletal myxoid chondrosarcoma is a rare sarcoma with low sensitivity to cytotoxic chemotherapy. " 12/18/2013 - "Anthracycline-based chemotherapy in extraskeletal myxoid chondrosarcoma: a retrospective study." 01/31/2023 - "Here we report a case involving a man in his late 60s with extraskeletal myxoid chondrosarcoma of the pelvis who responded to proton beam radiotherapy with intra-arterial chemotherapy and did not require surgery. "
2.	Therapeutics 01/01/2020 - "Patients with a smaller tumor size and extraskeletal myxoid chondrosarcoma subtype might benefit from this therapy more. " 09/01/2013 - "Some rare histological subtypes (alveolar soft part sarcoma, clear cell sarcoma, extraskeletal myxoid chondrosarcoma, solitary fibrous tumor) have shown promising sensitivity to molecular target agents in the metastatic setting, but the value of these therapies in the adjuvant one has not been studied yet. " 01/01/2022 - "Identification of Novel PGR-NR4A3 Fusion in Extraskeletal Myxoid Chondrosarcoma and Resultant Patient Benefit From Tamoxifen Therapy." 01/01/2020 - "Patients with mesenchymal, dedifferentiated, and extraskeletal myxoid chondrosarcoma subtypes and patients who received prior tyrosine kinase inhibitor therapy were excluded. " 07/01/1995 - "A patient with pulmonary metastatic extraskeletal myxoid chondrosarcoma (EMC), of unknown cause, responded dramatically to 16 months of therapy with interferon alfa-2b. "
3.	Radiotherapy 07/01/2018 - "Benefit of Radiotherapy in Extraskeletal Myxoid Chondrosarcoma: A Propensity Score Weighted Population-based Analysis of the SEER Database." 04/01/2018 - "The preoperative radiation therapy case was a patient with a grade 1 extraskeletal myxoid chondrosarcoma of the thigh. " 07/01/2018 - "Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignancy for which the role of radiotherapy is not well-defined. " 01/31/2023 - "Extraskeletal myxoid chondrosarcoma in the pelvis successfully treated with proton beam radiotherapy." 01/31/2023 - "Here we report a case involving a man in his late 60s with extraskeletal myxoid chondrosarcoma of the pelvis who responded to proton beam radiotherapy with intra-arterial chemotherapy and did not require surgery. "
4.	Chemoradiotherapy 12/24/2015 - "Unresectable Extraskeletal Myxoid Chondrosarcoma of the Neck: Early Tumor Response to Chemoradiotherapy."
5.	Neoadjuvant Therapy 12/11/2020 - "Locally recurrent extraskeletal myxoid chondrosarcoma of the shoulder: a case of complete neoadjuvant radiotherapy response."