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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 (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

Recent clinical studies

Etiology

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
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
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

Primary intra- and extradural extramedullary mesenchymal chondrosarcoma with isolated punctate calcification: case report and literature review.
Chen M, Lai Q
BMC Neurol 2022 Mar 23;22(1):112. doi: 10.1186/s12883-022-02645-x. PMID: 35321663Free 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
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
The clinical approach towards chondrosarcoma.
Gelderblom H, Hogendoorn PC, Dijkstra SD, van Rijswijk CS, Krol AD, Taminiau AH, Bovée JV
Oncologist 2008 Mar;13(3):320-9. doi: 10.1634/theoncologist.2007-0237. PMID: 18378543

Therapy

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
Results of sub-analysis of a phase 2 study on trabectedin treatment for extraskeletal myxoid chondrosarcoma and mesenchymal chondrosarcoma.
Morioka H, Takahashi S, Araki N, Sugiura H, Ueda T, Takahashi M, Yonemoto T, Hiraga H, Hiruma T, Kunisada T, Matsumine A, Susa M, Nakayama R, Nishimoto K, Kikuta K, Horiuchi K, Kawai A
BMC Cancer 2016 Jul 14;16:479. doi: 10.1186/s12885-016-2511-y. PMID: 27418251Free PMC Article
Sacral mesenchymal chondrosarcoma in childhood: a case report and review of the literature.
Küpeli S, Varan A, Gedikoğlu G, Büyükpamukçu M
Pediatr Hematol Oncol 2010 Oct;27(7):564-73. doi: 10.3109/08880018.2010.503333. PMID: 20677924
Mesenchymal chondrosarcoma of the orbit: a report of two cases and review of the literature.
Kaur A, Kishore P, Agrawal A, Gupta A
Orbit 2008;27(1):63-7. doi: 10.1080/01676830601169007. PMID: 18307151

Prognosis

Primary intra- and extradural extramedullary mesenchymal chondrosarcoma with isolated punctate calcification: case report and literature review.
Chen M, Lai Q
BMC Neurol 2022 Mar 23;22(1):112. doi: 10.1186/s12883-022-02645-x. PMID: 35321663Free PMC Article
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
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
The clinical approach towards chondrosarcoma.
Gelderblom H, Hogendoorn PC, Dijkstra SD, van Rijswijk CS, Krol AD, Taminiau AH, Bovée JV
Oncologist 2008 Mar;13(3):320-9. doi: 10.1634/theoncologist.2007-0237. PMID: 18378543

Clinical prediction guides

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
Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study.
Frezza AM, Cesari M, Baumhoer D, Biau D, Bielack S, Campanacci DA, Casanova J, Esler C, Ferrari S, Funovics PT, Gerrand C, Grimer R, Gronchi A, Haffner N, Hecker-Nolting S, Höller S, Jeys L, Jutte P, Leithner A, San-Julian M, Thorkildsen J, Vincenzi B, Windhager R, Whelan J
Eur J Cancer 2015 Feb;51(3):374-81. Epub 2014 Dec 16 doi: 10.1016/j.ejca.2014.11.007. PMID: 25529371

Recent systematic reviews

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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