Full Report

Format

Full Report
Summary (Text)
Summary (XML)

Send to:

Choose Destination

File
Clipboard
Collections

Extraskeletal myxoid chondrosarcoma

MedGen UID:: 220892
•Concept ID:: C1275278
•: Neoplastic Process

Synonym:	Chondrosarcoma, Extraskeletal Myxoid
SNOMED CT:	Extraskeletal myxoid chondrosarcoma (404079008); Chondroid sarcoma (404079008)
Modes of inheritance:	Not genetically inherited MedGen UID: 988794 •Concept ID: CN307044 • Finding Source: Orphanet clinical entity without genetic inheritance. See: This record Not genetically inherited (Orphanet)

Genes (locations): Gene(s) directly associated with this condition or phenotype.	NR4A3 (9q31.1); TAF15 (17q12)

Monarch Initiative:	MONDO:0012825
OMIM®:	612237
Orphanet:	ORPHA209916

Definition

Extraskeletal myxoid chondrosarcoma is a rare soft tissue neoplasm of chondroblastic origin. The tumors are most commonly found in middle-aged and elderly individuals, are more common among men, and are often detected as deep-seated lesions in the extremities. Despite their relatively low-grade malignancy, recurrence and metastasis may appear many years after the initial diagnosis. Histologic tissue section examination reveals a mixture of cellular and myxoid stromal components (Panagopoulos et al., 2002). [from OMIM]

Clinical features

From HPO

Chondrosarcoma

MedGen UID:: 3054
•Concept ID:: C0008479
•: Neoplastic Process

A slowly growing malignant neoplasm derived from cartilage cells.

See: Feature record | Search on this feature

Neoplasm
- Chondrosarcoma

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVExtraskeletal myxoid chondrosarcoma

Pathological process
- Disease
  - Neoplasm
    - Malignant neoplastic disease
      - Malignant soft tissue neoplasm
        Malignant Soft Tissue Tumor of Uncertain Differentiation
        Extraskeletal myxoid chondrosarcoma
        Adult myxoid chondrosarcoma
        Conventional Extraskeletal Myxoid Chondrosarcoma
        Intracranial extraskeletal myxoid chondrosarcoma
        Metastatic Extraskeletal Myxoid Chondrosarcoma
        Pediatric myxoid chondrosarcoma
        Recurrent Extraskeletal Myxoid Chondrosarcoma
        Refractory Extraskeletal Myxoid Chondrosarcoma

Follow this link to review classifications for Extraskeletal myxoid chondrosarcoma in Orphanet.

Professional guidelines

PubMed

Extraskeletal Myxoid Chondrosarcoma: Retrospective Case Series Examining Prognostic Factors, Treatment Approaches, and Oncologic Outcomes.

Brodsky CN, Bucala MD, Abdulfatah EM, Siegel GW
Am J Clin Oncol 2023 Apr 1;46(4):172-177. Epub 2023 Feb 24 doi: 10.1097/COC.0000000000000988. PMID: 36825763

Differential diagnosis of myxoid soft tissue tumors. Experience in the Clinical University Hospital of Valencia.

Alfaro-Cervelló C, Nieto G, Navarro S
Rev Esp Patol 2022 Jul-Sep;55(3):185-191. Epub 2019 Nov 4 doi: 10.1016/j.patol.2019.07.005. PMID: 35779885

SWI/SNF Complex-Deficient Soft Tissue Neoplasms: A Pattern-Based Approach to Diagnosis and Differential Diagnosis.

Agaimy A
Surg Pathol Clin 2019 Mar;12(1):149-163. doi: 10.1016/j.path.2018.10.006. PMID: 30709441

See all (5)

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Surgical outcomes of extraskeletal myxoid chondrosarcoma.

Ulucaköy C, Atalay İB, Yapar A, Kaptan AY, Bingöl İ, Doğan M, Ekşioğlu MF
Turk J Med Sci 2022 Aug;52(4):1183-1189. Epub 2022 Aug 10 doi: 10.55730/1300-0144.5422. PMID: 36326382 Free PMC Article

Musculoskeletal Tumors in Extremities Diagnosed During the Gestational Period: Case Series and Literature Review.

De La Calva Ceinos C, Copete I, Angulo M, Ferrás J, Amaya JV, Baixauli F
Acta Chir Orthop Traumatol Cech 2022;89(3):220-223. PMID: 35815490

Extraskeletal myxoid chondrosarcoma: Clinical features and overall survival.

Brown JM, Rakoczy K, Pretell-Mazzini J
Cancer Treat Res Commun 2022;31:100530. Epub 2022 Feb 5 doi: 10.1016/j.ctarc.2022.100530. PMID: 35144048

Soft tissue tumors associated with EWSR1 translocation.

Romeo S, Dei Tos AP
Virchows Arch 2010 Feb;456(2):219-34. doi: 10.1007/s00428-009-0854-3. PMID: 19936782

Extraskeletal myxoid chondrosarcoma.

McGrory JE, Rock MG, Nascimento AG, Oliveira AM
Clin Orthop Relat Res 2001 Jan;(382):185-90. doi: 10.1097/00003086-200101000-00025. PMID: 11153986

See all (69)

Diagnosis

Surgical outcomes of extraskeletal myxoid chondrosarcoma.

Extraskeletal myxoid chondrosarcoma: Clinical features and overall survival.

Brown JM, Rakoczy K, Pretell-Mazzini J
Cancer Treat Res Commun 2022;31:100530. Epub 2022 Feb 5 doi: 10.1016/j.ctarc.2022.100530. PMID: 35144048

Soft tissue tumors associated with EWSR1 translocation.

Romeo S, Dei Tos AP
Virchows Arch 2010 Feb;456(2):219-34. doi: 10.1007/s00428-009-0854-3. PMID: 19936782

Primary malignant myoepithelioma of the distal femur.

Alberghini M, Pasquinelli G, Zanella L, Pignatti G, Benini S, Bacchini P, Bertoni F
APMIS 2007 Apr;115(4):376-80. doi: 10.1111/j.1600-0463.2007.apm_569.x. PMID: 17504307

Immunohistochemical staining for KIT (CD117) in soft tissue sarcomas is very limited in distribution.

Hornick JL, Fletcher CD
Am J Clin Pathol 2002 Feb;117(2):188-93. doi: 10.1309/LX9U-F7P0-UWDH-8Y6R. PMID: 11865845

See all (185)

Therapy

Pazopanib for treatment of advanced extraskeletal myxoid chondrosarcoma: a multicentre, single-arm, phase 2 trial.

Stacchiotti S, Ferrari S, Redondo A, Hindi N, Palmerini E, Vaz Salgado MA, Frezza AM, Casali PG, Gutierrez A, Lopez-Pousa A, Grignani G, Italiano A, LeCesne A, Dumont S, Blay JY, Penel N, Bernabeu D, de Alava E, Karanian M, Morosi C, Brich S, Dagrada GP, Vallacchi V, Castelli C, Brenca M, Racanelli D, Maestro R, Collini P, Cruz J, Martin-Broto J
Lancet Oncol 2019 Sep;20(9):1252-1262. Epub 2019 Jul 19 doi: 10.1016/S1470-2045(19)30319-5. PMID: 31331701

Clinical benefit of antiangiogenic therapy in advanced and metastatic chondrosarcoma.

Jones RL, Katz D, Loggers ET, Davidson D, Rodler ET, Pollack SM
Med Oncol 2017 Aug 29;34(10):167. doi: 10.1007/s12032-017-1030-2. PMID: 28852958 Free PMC Article

Activity of sunitinib in extraskeletal myxoid chondrosarcoma.

Stacchiotti S, Pantaleo MA, Astolfi A, Dagrada GP, Negri T, Dei Tos AP, Indio V, Morosi C, Gronchi A, Colombo C, Conca E, Toffolatti L, Tazzari M, Crippa F, Maestro R, Pilotti S, Casali PG
Eur J Cancer 2014 Jun;50(9):1657-64. Epub 2014 Apr 2 doi: 10.1016/j.ejca.2014.03.013. PMID: 24703573

Emerging therapies for adult soft tissue sarcoma.

Radaelli S, Stacchiotti S, Casali PG, Gronchi A
Expert Rev Anticancer Ther 2014 Jun;14(6):689-704. Epub 2014 Feb 20 doi: 10.1586/14737140.2014.885840. PMID: 24555529

A myxoid chondrosarcoma associated with an anti-Hu-positive paraneoplastic encephalomyelitis.

Hoosien M, Vredenburgh J, Lanfranco J, Ochoa R, Robinson P, Martinez R, Singer C, Silva O
J Neurooncol 2011 Jan;101(1):135-9. Epub 2010 May 5 doi: 10.1007/s11060-010-0216-7. PMID: 20443130

See all (19)

Prognosis

Surgical outcomes of extraskeletal myxoid chondrosarcoma.

SWI/SNF Complex-Deficient Soft Tissue Neoplasms: A Pattern-Based Approach to Diagnosis and Differential Diagnosis.

Agaimy A
Surg Pathol Clin 2019 Mar;12(1):149-163. doi: 10.1016/j.path.2018.10.006. PMID: 30709441

Immunohistochemical staining for KIT (CD117) in soft tissue sarcomas is very limited in distribution.

Hornick JL, Fletcher CD
Am J Clin Pathol 2002 Feb;117(2):188-93. doi: 10.1309/LX9U-F7P0-UWDH-8Y6R. PMID: 11865845

Extraskeletal myxoid chondrosarcoma.

McGrory JE, Rock MG, Nascimento AG, Oliveira AM
Clin Orthop Relat Res 2001 Jan;(382):185-90. doi: 10.1097/00003086-200101000-00025. PMID: 11153986

Oncogenic conversion of a novel orphan nuclear receptor by chromosome translocation.

Labelle Y, Zucman J, Stenman G, Kindblom LG, Knight J, Turc-Carel C, Dockhorn-Dworniczak B, Mandahl N, Desmaze C, Peter M
Hum Mol Genet 1995 Dec;4(12):2219-26. doi: 10.1093/hmg/4.12.2219. PMID: 8634690

See all (86)