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Mesenchymal Chondrosarcoma of Bone

MedGen UID:
317901
•Concept ID:
C1708980
•
Neoplastic Process
Synonym: Mesenchymal Chondrosarcoma of the Bone

Definition

A morphologic variant of chondrosarcoma arising from the bone. 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]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMesenchymal Chondrosarcoma of Bone

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
Advances in the management of primary bone sarcomas of the skull base.
Edem I, DeMonte F, Raza SM
J Neurooncol 2020 Dec;150(3):393-403. Epub 2020 Apr 18 doi: 10.1007/s11060-020-03497-6. PMID: 32306199
The clinical management of chondrosarcoma.
Riedel RF, Larrier N, Dodd L, Kirsch D, Martinez S, Brigman BE
Curr Treat Options Oncol 2009 Apr;10(1-2):94-106. Epub 2009 Feb 24 doi: 10.1007/s11864-009-0088-2. PMID: 19238552

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
The 2020 WHO Classification of Tumors of Bone: An Updated Review.
Choi JH, Ro JY
Adv Anat Pathol 2021 May 1;28(3):119-138. doi: 10.1097/PAP.0000000000000293. PMID: 33480599
Biology of Bone Sarcomas and New Therapeutic Developments.
Brown HK, Schiavone K, Gouin F, Heymann MF, Heymann D
Calcif Tissue Int 2018 Feb;102(2):174-195. Epub 2017 Dec 13 doi: 10.1007/s00223-017-0372-2. PMID: 29238848Free PMC Article
Cartilage-forming tumors.
Qasem SA, DeYoung BR
Semin Diagn Pathol 2014 Jan;31(1):10-20. Epub 2014 Jan 23 doi: 10.1053/j.semdp.2014.01.006. PMID: 24680178
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Diagnosis

Malignant Cartilage-Forming Tumors.
Hameed M
Surg Pathol Clin 2021 Dec;14(4):605-617. Epub 2021 Oct 7 doi: 10.1016/j.path.2021.06.005. PMID: 34742483
The 2020 WHO Classification of Tumors of Bone: An Updated Review.
Choi JH, Ro JY
Adv Anat Pathol 2021 May 1;28(3):119-138. doi: 10.1097/PAP.0000000000000293. PMID: 33480599
The epigenomics of sarcoma.
Nacev BA, Jones KB, Intlekofer AM, Yu JSE, Allis CD, Tap WD, Ladanyi M, Nielsen TO
Nat Rev Cancer 2020 Oct;20(10):608-623. Epub 2020 Aug 11 doi: 10.1038/s41568-020-0288-4. PMID: 32782366Free 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
Cartilage-forming tumors.
Qasem SA, DeYoung BR
Semin Diagn Pathol 2014 Jan;31(1):10-20. Epub 2014 Jan 23 doi: 10.1053/j.semdp.2014.01.006. PMID: 24680178

Therapy

Systemic Therapy for Chondrosarcoma.
Rock A, Ali S, Chow WA
Curr Treat Options Oncol 2022 Feb;23(2):199-209. Epub 2022 Feb 21 doi: 10.1007/s11864-022-00951-7. PMID: 35190971
The biology and management of cartilaginous tumors: a role for targeting isocitrate dehydrogenase.
Tinoco G, Wilky BA, Paz-Mejia A, Rosenberg A, Trent JC
Am Soc Clin Oncol Educ Book 2015:e648-55. doi: 10.14694/EdBook_AM.2015.35.e648. PMID: 25993236
Anti-angiogenic therapy, a new player in the field of sarcoma treatment.
Versleijen-Jonkers YM, Vlenterie M, van de Luijtgaarden AC, van der Graaf WT
Crit Rev Oncol Hematol 2014 Aug;91(2):172-85. Epub 2014 Feb 15 doi: 10.1016/j.critrevonc.2014.02.001. PMID: 24613529
The clinical management of chondrosarcoma.
Riedel RF, Larrier N, Dodd L, Kirsch D, Martinez S, Brigman BE
Curr Treat Options Oncol 2009 Apr;10(1-2):94-106. Epub 2009 Feb 24 doi: 10.1007/s11864-009-0088-2. PMID: 19238552
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Prognosis

Malignant Cartilage-Forming Tumors.
Hameed M
Surg Pathol Clin 2021 Dec;14(4):605-617. Epub 2021 Oct 7 doi: 10.1016/j.path.2021.06.005. PMID: 34742483
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
The 2020 WHO Classification of Tumors of Bone: An Updated Review.
Choi JH, Ro JY
Adv Anat Pathol 2021 May 1;28(3):119-138. doi: 10.1097/PAP.0000000000000293. PMID: 33480599
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

Clinical prediction guides

Molecular imaging of sarcomas with FDG PET.
Mendoza H, Nosov A, Pandit-Taskar N
Skeletal Radiol 2023 Mar;52(3):461-475. Epub 2022 Sep 29 doi: 10.1007/s00256-022-04182-7. PMID: 36173459
Systemic Therapy for Chondrosarcoma.
Rock A, Ali S, Chow WA
Curr Treat Options Oncol 2022 Feb;23(2):199-209. Epub 2022 Feb 21 doi: 10.1007/s11864-022-00951-7. PMID: 35190971
Malignant Cartilage-Forming Tumors.
Hameed M
Surg Pathol Clin 2021 Dec;14(4):605-617. Epub 2021 Oct 7 doi: 10.1016/j.path.2021.06.005. PMID: 34742483
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
Multicentric chondrosarcomas.
Damron TA, Sim FH, Unni KK
Clin Orthop Relat Res 1996 Jul;(328):211-9. doi: 10.1097/00003086-199607000-00033. PMID: 8653959

Recent systematic reviews

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
Towards a new understanding and classification of chondrogenic neoplasias of the skeleton--biochemistry and cell biology of chondrosarcoma and its variants.
Aigner T
Virchows Arch 2002 Sep;441(3):219-30. Epub 2002 May 1 doi: 10.1007/s00428-002-0641-x. PMID: 12242518

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