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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
Small round blue cell tumors of the sinonasal tract: a differential diagnosis approach.
Thompson LD
Mod Pathol 2017 Jan;30(s1):S1-S26. doi: 10.1038/modpathol.2016.119. PMID: 28060373

Curated

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

Recent clinical studies

Etiology

Mesenchymal chondrosarcoma: An Australian multi-centre cohort study.
Strach MC, Grimison PS, Hong A, Boyle R, Stalley P, Karim R, Connolly EA, Bae S, Desai J, Crowe P, Singhal N, Bhadri VA
Cancer Med 2023 Jan;12(1):368-378. Epub 2022 May 23 doi: 10.1002/cam4.4849. PMID: 35603739Free PMC Article
Mesenchymal Chondrosarcoma-A Retrospective study.
Beena D, Kattoor J, Mathews A, P Nair S, M V, T P, N G
Gulf J Oncolog 2021 Jan;1(35):54-58. PMID: 33716213
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
Mesenchymal chondrosarcoma: imaging features and clinical findings.
Ghafoor S, Hameed MR, Tap WD, Hwang S
Skeletal Radiol 2021 Feb;50(2):333-341. Epub 2020 Jul 30 doi: 10.1007/s00256-020-03558-x. PMID: 32734374Free PMC Article
Mesenchymal chondrosarcoma of the orbit: imaging features of CT and MRI.
Tsuchiya M, Masui T, Otsuki Y, Sakahara H
Br J Radiol 2018 Oct;91(1090):20170579. Epub 2018 Jul 25 doi: 10.1259/bjr.20170579. PMID: 29975155Free PMC Article

Diagnosis

Mesenchymal Chondrosarcoma-A Retrospective study.
Beena D, Kattoor J, Mathews A, P Nair S, M V, T P, N G
Gulf J Oncolog 2021 Jan;1(35):54-58. PMID: 33716213
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

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
Mesenchymal chondrosarcoma: A Japanese Musculoskeletal Oncology Group (JMOG) study on 57 patients.
Tsuda Y, Ogura K, Hakozaki M, Kikuta K, Ae K, Tsuchiya H, Iwata S, Ueda T, Kawano H, Kawai A
J Surg Oncol 2017 May;115(6):760-767. doi: 10.1002/jso.24567. PMID: 29044531
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: 28852958Free PMC Article
Trabectedin for Soft Tissue Sarcoma: Current Status and Future Perspectives.
Gordon EM, Sankhala KK, Chawla N, Chawla SP
Adv Ther 2016 Jul;33(7):1055-71. Epub 2016 May 27 doi: 10.1007/s12325-016-0344-3. PMID: 27234989Free PMC Article
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

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
NKX3.1 immunoreactivity is not identified in mesenchymal chondrosarcoma: a 25-case cohort study.
Chen W, Hornick JL, Fletcher CDM
Histopathology 2021 Jan;78(2):334-337. Epub 2020 Oct 19 doi: 10.1111/his.14231. PMID: 32779239
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
The Differences Between Intracranial Mesenchymal Chondrosarcoma and Conventional Chondrosarcoma in Clinical Features and Outcomes.
Ma X, Meng G, Wang K, Li D, Wang L, Li H, Zhang J, Zhang L, Wu Z
World Neurosurg 2019 Feb;122:e1078-e1082. Epub 2018 Nov 9 doi: 10.1016/j.wneu.2018.10.230. PMID: 30415056
Ewing Sarcoma and the History of Similar and Possibly Related Small Round Cell Tumors: From Whence Have We Come and Where are We Going?
Kilpatrick SE, Reith JD, Rubin B
Adv Anat Pathol 2018 Sep;25(5):314-326. doi: 10.1097/PAP.0000000000000203. PMID: 29911999

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

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    Curated

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

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