U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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): 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

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

Curated

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

Recent clinical studies

Etiology

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
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
Update on chondrosarcomas.
Chow WA
Curr Opin Oncol 2007 Jul;19(4):371-6. doi: 10.1097/CCO.0b013e32812143d9. PMID: 17545802
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
The role of cytogenetics in the classification of soft tissue tumours.
Dei Tos AP, Dal Cin P
Virchows Arch 1997 Aug;431(2):83-94. doi: 10.1007/s004280050073. PMID: 9293889

Diagnosis

PRAME immunohistochemistry in soft tissue tumors and mimics: a study of 350 cases highlighting its imperfect specificity but potentially useful diagnostic applications.
Cammareri C, Beltzung F, Michal M, Vanhersecke L, Coindre JM, Velasco V, Le Loarer F, Vergier B, Perret R
Virchows Arch 2023 Aug;483(2):145-156. Epub 2023 Jul 21 doi: 10.1007/s00428-023-03606-6. PMID: 37477762
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
Extraskeletal Myxoid Chondrosarcoma with Molecularly Confirmed Diagnosis: A Multicenter Retrospective Study Within the Italian Sarcoma Group.
Paioli A, Stacchiotti S, Campanacci D, Palmerini E, Frezza AM, Longhi A, Radaelli S, Donati DM, Beltrami G, Bianchi G, Barisella M, Righi A, Benini S, Fiore M, Picci P, Gronchi A
Ann Surg Oncol 2021 Feb;28(2):1142-1150. Epub 2020 Jun 22 doi: 10.1245/s10434-020-08737-7. PMID: 32572850
The diversity of soft tissue tumours with EWSR1 gene rearrangements: a review.
Fisher C
Histopathology 2014 Jan;64(1):134-50. Epub 2013 Nov 6 doi: 10.1111/his.12269. PMID: 24320889
Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 18 cases.
Okamoto S, Hisaoka M, Ishida T, Imamura T, Kanda H, Shimajiri S, Hashimoto H
Hum Pathol 2001 Oct;32(10):1116-24. doi: 10.1053/hupa.2001.28226. PMID: 11679947

Therapy

Results of a prospective phase 2 study of pazopanib in patients with surgically unresectable or metastatic chondrosarcoma.
Chow W, Frankel P, Ruel C, Araujo DM, Milhem M, Okuno S, Hartner L, Undevia S, Staddon A
Cancer 2020 Jan 1;126(1):105-111. Epub 2019 Sep 11 doi: 10.1002/cncr.32515. PMID: 31509242
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
Adjuvant therapy for high-risk soft tissue sarcoma in the adult.
Gronchi A, Casali PG
Curr Treat Options Oncol 2013 Sep;14(3):415-24. doi: 10.1007/s11864-013-0243-7. PMID: 23775297
Why do patients with low-grade soft tissue sarcoma die?
Canter RJ, Qin LX, Ferrone CR, Maki RG, Singer S, Brennan MF
Ann Surg Oncol 2008 Dec;15(12):3550-60. Epub 2008 Oct 1 doi: 10.1245/s10434-008-0163-0. PMID: 18830667Free PMC Article

Prognosis

Extraskeletal Myxoid Chondrosarcomas: The Uncommon Clinicopathologic Manifestations and Significance of TAF15::NR4A3 Fusion.
Huang SC, Lee JC, Hsu YC, Tsai JW, Kao YC, Hsieh TH, Chang YM, Chang KC, Wu PS, Chen PC, Chen CH, Chang CD, Lee PH, Tai HC, Liu TT, Wen MC, Li WS, Yu SC, Wang JC, Huang HY
Mod Pathol 2023 Jul;36(7):100161. Epub 2023 Mar 21 doi: 10.1016/j.modpat.2023.100161. PMID: 36948401
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
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
Update on chondrosarcomas.
Chow WA
Curr Opin Oncol 2007 Jul;19(4):371-6. doi: 10.1097/CCO.0b013e32812143d9. PMID: 17545802
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

Clinical prediction guides

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
Analysis of clinicopathologic features and expression of NR4A3 in sinonasal acinic cell carcinoma.
Wang H, Zhai C, Zhang C, Liu Q, Zhang H, Sun X, Lin L, Yu H, Wang D
Mod Pathol 2022 May;35(5):594-600. Epub 2021 Dec 6 doi: 10.1038/s41379-021-00959-8. PMID: 34873305
SWI/SNF complex-deficient soft tissue neoplasms: An update.
Schaefer IM, Hornick JL
Semin Diagn Pathol 2021 May;38(3):222-231. Epub 2020 Jun 5 doi: 10.1053/j.semdp.2020.05.005. PMID: 32646614Free PMC Article
Molecular and Clinicopathologic Heterogeneity of Intracranial Tumors Mimicking Extraskeletal Myxoid Chondrosarcoma.
Velz J, Agaimy A, Frontzek K, Neidert MC, Bozinov O, Wagner U, Fritz C, Coras R, Hofer S, Bode-Lesniewska B, Rushing E
J Neuropathol Exp Neurol 2018 Aug 1;77(8):727-735. doi: 10.1093/jnen/nly050. PMID: 29924341
The role of cytogenetics in the classification of soft tissue tumours.
Dei Tos AP, Dal Cin P
Virchows Arch 1997 Aug;431(2):83-94. doi: 10.1007/s004280050073. PMID: 9293889

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

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

    Reviews

    Related information

    • Gene
      Related information in NCBI Gene
    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • OMIM(Genes)
      OMIM records containing genes associated with phenotypes registered in MedGen
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...