U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Mesenchymal chondrosarcoma

MedGen UID:
104904
•Concept ID:
C0206637
•
Neoplastic Process
Synonyms: Chondrosarcoma, Mesenchymal; Chondrosarcomas, Mesenchymal; Mesenchymal Chondrosarcoma; Mesenchymal Chondrosarcomas
SNOMED CT: Mesenchymal chondrosarcoma (1290898006); Mesenchymal chondrosarcoma (56565002)
 
Monarch Initiative: MONDO:0006853

Definition

A morphologic variant of chondrosarcoma arising from bone and soft tissue. It is characterized by the presence of malignant small round cells, biphasic growth pattern, and well differentiated hyaline cartilage. Clinical presentation includes pain and swelling. The clinical course is aggressive, with local recurrences and distant metastases. [from NCI]

Professional guidelines

PubMed

Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options.
Tlemsani C, Larousserie F, De Percin S, Audard V, Hadjadj D, Chen J, Biau D, Anract P, Terris B, Goldwasser F, Pasmant E, Boudou-Rouquette P
Int J Mol Sci 2023 Jan 10;24(2) doi: 10.3390/ijms24021361. PMID: 36674874Free PMC Article
Small round cell lesions of the bone: Diagnostic approach, differential diagnoses and impact on treatment.
Rekhi B, Mridha A, Kattoor J
Indian J Pathol Microbiol 2019 Apr-Jun;62(2):199-205. doi: 10.4103/IJPM.IJPM_675_18. PMID: 30971540
Clinical presentation and management of gastro-intestinal and pancreatic secondary metastatic tumors.
Fragulidis GP, Vezakis A, Chondrogiannis K, Mellou A, Melemeni A, Polydorou A
J BUON 2015 Jul-Aug;20(4):1009-14. PMID: 26416049

Curated

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

Recent clinical studies

Etiology

Chondrosarcoma.
Weinschenk RC, Wang WL, Lewis VO
J Am Acad Orthop Surg 2021 Jul 1;29(13):553-562. doi: 10.5435/JAAOS-D-20-01188. PMID: 33595238
Adolescent and Young Adult Oral Maxillofacial Tumors: A Single-Institution Case Series and Literature Review.
George AP, Markiewicz MR, Garzon S, Choi DK
J Adolesc Young Adult Oncol 2020 Apr;9(2):307-312. Epub 2019 Dec 20 doi: 10.1089/jayao.2019.0111. PMID: 31859579Free PMC Article
Integrating Morphology and Genetics in the Diagnosis of Cartilage Tumors.
de Andrea CE, San-Julian M, Bovée JVMG
Surg Pathol Clin 2017 Sep;10(3):537-552. doi: 10.1016/j.path.2017.04.005. PMID: 28797501
Mesenchymal chondrosarcoma of the maxilla: case report and literature review.
Tien N, Chaisuparat R, Fernandes R, Sarlani E, Papadimitriou JC, Ord RA, Nikitakis NG
J Oral Maxillofac Surg 2007 Jun;65(6):1260-6. doi: 10.1016/j.joms.2005.11.074. PMID: 17517320
Immunohistochemistry of small round-cell tumors.
Devoe K, Weidner N
Semin Diagn Pathol 2000 Aug;17(3):216-24. PMID: 10968707

Diagnosis

Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
Bone Pathology for Hematopathologists.
Peker D, Wei S, Siegal GP
Surg Pathol Clin 2019 Sep;12(3):831-847. Epub 2019 May 22 doi: 10.1016/j.path.2019.03.007. PMID: 31352990
Extraskeletal Mesenchymal Chondrosarcoma.
Arora K, Riddle ND
Arch Pathol Lab Med 2018 Nov;142(11):1421-1424. doi: 10.5858/arpa.2017-0109-RS. PMID: 30407856
Integrating Morphology and Genetics in the Diagnosis of Cartilage Tumors.
de Andrea CE, San-Julian M, Bovée JVMG
Surg Pathol Clin 2017 Sep;10(3):537-552. doi: 10.1016/j.path.2017.04.005. PMID: 28797501
Intravascular mesenchymal chondrosarcoma.
Simon B, Chandramohan A, Eapen A, Nayak S, Jeyaraj V
J Vasc Interv Radiol 2014 Dec;25(12):1937. Epub 2014 Nov 20 doi: 10.1016/j.jvir.2014.07.030. PMID: 25457141

Therapy

Clinical Efficacy of Olaparib in IDH1/IDH2-Mutant Mesenchymal Sarcomas.
Eder JP, Doroshow DB, Do KT, Keedy VL, Sklar JS, Glazer P, Bindra R, Shapiro GI
JCO Precis Oncol 2021 Nov;5:466-472. doi: 10.1200/PO.20.00247. PMID: 34994649Free PMC Article
Time lapse analysis of tumor response in patients with soft tissue sarcoma treated with trabectedin: A pooled analysis of two phase II clinical trials.
Endo M, Takahashi S, Araki N, Sugiura H, Ueda T, Yonemoto T, Takahashi M, Morioka H, Hiraga H, Hiruma T, Kunisada T, Matsumine A, Goda K, Kawai A
Cancer Med 2020 Jun;9(11):3656-3667. Epub 2020 Mar 27 doi: 10.1002/cam4.2991. PMID: 32220056Free PMC Article
Mesenchymal chondrosarcoma metastasising to the pancreas.
Paasch C, De Santo G, Boettge KR, Strik MW
BMJ Case Rep 2018 Dec 31;11(1) doi: 10.1136/bcr-2018-226369. PMID: 30598468Free PMC Article
Mesenchymal chondrosarcoma and simulating lesions of the orbit.
Koeller KK
Radiol Clin North Am 1999 Jan;37(1):203-17, xii. doi: 10.1016/s0033-8389(05)70088-9. PMID: 10026739
Mesenchymal chondrosarcoma of the orbit.
Sevel D
Br J Ophthalmol 1974 Oct;58(10):882-7. doi: 10.1136/bjo.58.10.882. PMID: 4451652Free PMC Article

Prognosis

Chondrosarcoma.
Weinschenk RC, Wang WL, Lewis VO
J Am Acad Orthop Surg 2021 Jul 1;29(13):553-562. doi: 10.5435/JAAOS-D-20-01188. PMID: 33595238
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
Bone Pathology for Hematopathologists.
Peker D, Wei S, Siegal GP
Surg Pathol Clin 2019 Sep;12(3):831-847. Epub 2019 May 22 doi: 10.1016/j.path.2019.03.007. PMID: 31352990
Extraskeletal Mesenchymal Chondrosarcoma.
Arora K, Riddle ND
Arch Pathol Lab Med 2018 Nov;142(11):1421-1424. doi: 10.5858/arpa.2017-0109-RS. PMID: 30407856
Intravascular mesenchymal chondrosarcoma.
Simon B, Chandramohan A, Eapen A, Nayak S, Jeyaraj V
J Vasc Interv Radiol 2014 Dec;25(12):1937. Epub 2014 Nov 20 doi: 10.1016/j.jvir.2014.07.030. PMID: 25457141

Clinical prediction guides

Fusion of the Genes for Interferon Regulatory Factor 2 Binding Protein 2 (IRF2BP2) and Caudal Type Homeobox 1 (CDX1) in a Chondrogenic Tumor.
Panagopoulos I, Gorunova L, Lobmaier I, Heim S
In Vivo 2023 Nov-Dec;37(6):2459-2463. doi: 10.21873/invivo.13352. PMID: 37905608Free PMC Article
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
Pancreatic involvement by mesenchymal chondrosarcoma harboring the HEY1-NCOA2 gene fusion.
Cohen JN, Solomon DA, Horvai AE, Kakar S
Hum Pathol 2016 Dec;58:35-40. Epub 2016 Aug 18 doi: 10.1016/j.humpath.2016.07.026. PMID: 27544802
Mesenchymal chondrosarcoma--observations on 30 new cases.
Salvador AH, Beabout JW, Dahlin DC
Cancer 1971 Sep;28(3):605-15. doi: 10.1002/1097-0142(197109)28:3<605::aid-cncr2820280312>3.0.co;2-r. PMID: 5096926
Mesenchymal chondrosarcoma. Further observations on a new entity.
DAHLIN DC, HENDERSON ED
Cancer 1962 Mar-Apr;15:410-7. doi: 10.1002/1097-0142(196203/04)15:2<410::aid-cncr2820150225>3.0.co;2-l. PMID: 13883114

Recent systematic reviews

Extra-skeletal intracranial mesenchymal chondrosarcoma: systematic-literature review.
Kumarasamy S, Garg K, Garg A, Sharma MC, Singh M, Chandra PS, Kale SS
Childs Nerv Syst 2024 Sep;40(9):2723-2733. Epub 2024 May 19 doi: 10.1007/s00381-024-06452-2. PMID: 38762839
Primary central nervous system sarcomas in adults: A systematic review.
Haider AS, Palmisciano P, Sagoo NS, Bin Alamer O, El Ahmadieh TY, Pan E, Garzon-Muvdi T
Clin Neurol Neurosurg 2022 Mar;214:107127. Epub 2022 Jan 12 doi: 10.1016/j.clineuro.2022.107127. PMID: 35151057
Mesenchymal chondrosarcoma of bone and soft tissue: a systematic review of 107 patients in the past 20 years.
Xu J, Li D, Xie L, Tang S, Guo W
PLoS One 2015;10(4):e0122216. Epub 2015 Apr 7 doi: 10.1371/journal.pone.0122216. PMID: 25849226Free PMC Article

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

    Consumer resources

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • 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...