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Chondrosarcoma, Grade 3

MedGen UID:
824654
•Concept ID:
C3838808
•
Neoplastic Process
Synonyms: Chondrosarcoma, grade 3; Grade III Chondrosarcoma; High Grade Chondrosarcoma; High-Grade Chondrosarcoma
SNOMED CT: Chondrosarcoma, grade 3 (703700004)

Definition

A high-grade chondrosarcoma arising in the medulla of bone or within the cartilaginous cap of a pre-existing osteochondroma. It is characterized by the presence of increased cellularity and a greater degree of nuclear atypia, hyperchromasia, and mitotic activity as compared to grade 2 chondrosarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChondrosarcoma, Grade 3

Professional guidelines

PubMed

Clinical features and treatment outcomes of dedifferentiated and grade 3 chondrosarcoma: A multi-institutional study.
Kozawa E, Nishida Y, Kawai A, Hayakawa K, Setsu N, Kawashima H, Iwata S, Tsuchiya H, Tsukushi S, Takenaka S, Imanishi J, Baba I, Nagano A, Morii T, Shirai T, Shimizu K, Kawano H
Cancer Sci 2022 Jul;113(7):2397-2408. Epub 2022 May 18 doi: 10.1111/cas.15382. PMID: 35485870Free PMC Article

Recent clinical studies

Etiology

Bone and soft tissue tumors at the borderlands of malignancy.
Crim J, Layfield LJ
Skeletal Radiol 2023 Mar;52(3):379-392. Epub 2022 Jun 29 doi: 10.1007/s00256-022-04099-1. PMID: 35767018
Chondrosarcoma of the hands and feet.
Kask G, Laitinen MK, Stevenson J, Evans S, Jeys LM, Parry MC
Bone Joint J 2021 Mar;103-B(3):562-568. doi: 10.1302/0301-620X.103B3.BJJ-2020-1337.R1. PMID: 33641425
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362
Dedifferentiated chondrosarcoma.
Mercuri M, Picci P, Campanacci L, Rulli E
Skeletal Radiol 1995 Aug;24(6):409-16. doi: 10.1007/BF00941235. PMID: 7481896

Diagnosis

Bone and soft tissue tumors at the borderlands of malignancy.
Crim J, Layfield LJ
Skeletal Radiol 2023 Mar;52(3):379-392. Epub 2022 Jun 29 doi: 10.1007/s00256-022-04099-1. PMID: 35767018
SWI/SNF-deficient thoraco-pulmonary neoplasms.
Sesboue C, Le Loarer F
Semin Diagn Pathol 2021 May;38(3):183-194. Epub 2021 Jan 12 doi: 10.1053/j.semdp.2020.12.002. PMID: 33451916
Chondrosarcomas revisited.
Mavrogenis AF, Gambarotti M, Angelini A, Palmerini E, Staals EL, Ruggieri P, Papagelopoulos PJ
Orthopedics 2012 Mar 7;35(3):e379-90. doi: 10.3928/01477447-20120222-30. PMID: 22385450
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362
Dedifferentiated chondrosarcoma.
Mercuri M, Picci P, Campanacci L, Rulli E
Skeletal Radiol 1995 Aug;24(6):409-16. doi: 10.1007/BF00941235. PMID: 7481896

Therapy

Can low-grade chondrosarcoma in flat bones be treated with intralesional curettage and cryotherapy?
Park K, Krumme J, Adebayo M, Adams BW, Henshaw RM
J Surg Oncol 2023 Mar;127(3):473-479. Epub 2022 Oct 17 doi: 10.1002/jso.27123. PMID: 36250903
Phase I Study of the Mutant IDH1 Inhibitor Ivosidenib: Safety and Clinical Activity in Patients With Advanced Chondrosarcoma.
Tap WD, Villalobos VM, Cote GM, Burris H, Janku F, Mir O, Beeram M, Wagner AJ, Jiang L, Wu B, Choe S, Yen K, Gliser C, Fan B, Agresta S, Pandya SS, Trent JC
J Clin Oncol 2020 May 20;38(15):1693-1701. Epub 2020 Mar 24 doi: 10.1200/JCO.19.02492. PMID: 32208957Free PMC Article
Biological aspects of chondrosarcoma: Leaps and hurdles.
Mery B, Espenel S, Guy JB, Rancoule C, Vallard A, Aloy MT, Rodriguez-Lafrasse C, Magné N
Crit Rev Oncol Hematol 2018 Jun;126:32-36. Epub 2018 Mar 22 doi: 10.1016/j.critrevonc.2018.03.009. PMID: 29759564
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Prognosis

Clinical features and treatment outcomes of dedifferentiated and grade 3 chondrosarcoma: A multi-institutional study.
Kozawa E, Nishida Y, Kawai A, Hayakawa K, Setsu N, Kawashima H, Iwata S, Tsuchiya H, Tsukushi S, Takenaka S, Imanishi J, Baba I, Nagano A, Morii T, Shirai T, Shimizu K, Kawano H
Cancer Sci 2022 Jul;113(7):2397-2408. Epub 2022 May 18 doi: 10.1111/cas.15382. PMID: 35485870Free PMC Article
Chondrosarcoma of the hands and feet.
Kask G, Laitinen MK, Stevenson J, Evans S, Jeys LM, Parry MC
Bone Joint J 2021 Mar;103-B(3):562-568. doi: 10.1302/0301-620X.103B3.BJJ-2020-1337.R1. PMID: 33641425
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362
Dedifferentiated chondrosarcoma.
Mercuri M, Picci P, Campanacci L, Rulli E
Skeletal Radiol 1995 Aug;24(6):409-16. doi: 10.1007/BF00941235. PMID: 7481896

Clinical prediction guides

Development and validation of a nomogram for predicting prognosis of high-grade chondrosarcoma: A surveillance, epidemiology, and end results-based population analysis.
Sun Y, Ouyang C, Zhang Y, Li Y, Liu Y, Jiang M, Nong L, Gao G
J Orthop Surg (Hong Kong) 2023 May-Aug;31(2):10225536231174255. doi: 10.1177/10225536231174255. PMID: 37147017
Can low-grade chondrosarcoma in flat bones be treated with intralesional curettage and cryotherapy?
Park K, Krumme J, Adebayo M, Adams BW, Henshaw RM
J Surg Oncol 2023 Mar;127(3):473-479. Epub 2022 Oct 17 doi: 10.1002/jso.27123. PMID: 36250903
Clinical Characteristics, Prognostic Factor and a Novel Dynamic Prediction Model for Overall Survival of Elderly Patients With Chondrosarcoma: A Population-Based Study.
Tong Y, Cui Y, Jiang L, Pi Y, Gong Y, Zhao D
Front Public Health 2022;10:901680. Epub 2022 Jun 30 doi: 10.3389/fpubh.2022.901680. PMID: 35844853Free PMC Article
Imaging features of laryngeal chondrosarcomas: A case series and systematic review.
Baba A, Kurokawa R, Kurokawa M, Ota Y, Lobo R, Srinivasan A
J Neuroimaging 2022 Mar;32(2):213-222. Epub 2022 Jan 12 doi: 10.1111/jon.12963. PMID: 35019171
Chondrosarcoma of the hands and feet.
Kask G, Laitinen MK, Stevenson J, Evans S, Jeys LM, Parry MC
Bone Joint J 2021 Mar;103-B(3):562-568. doi: 10.1302/0301-620X.103B3.BJJ-2020-1337.R1. PMID: 33641425

Recent systematic reviews

Efficacy and safety of carbon ion radiotherapy for bone sarcomas: a systematic review and meta-analysis.
Dong M, Liu R, Zhang Q, Luo H, Wang D, Wang Y, Chen J, Ou Y, Wang X
Radiat Oncol 2022 Oct 25;17(1):172. doi: 10.1186/s13014-022-02089-0. PMID: 36284346Free PMC Article
Imaging features of laryngeal chondrosarcomas: A case series and systematic review.
Baba A, Kurokawa R, Kurokawa M, Ota Y, Lobo R, Srinivasan A
J Neuroimaging 2022 Mar;32(2):213-222. Epub 2022 Jan 12 doi: 10.1111/jon.12963. PMID: 35019171
Treatment strategies for central low-grade chondrosarcoma of long bones: a systematic review of the literature and meta-analysis.
Shemesh SS, Acevedo-Nieves JD, Pretell-Mazzini J
Musculoskelet Surg 2018 Aug;102(2):95-109. Epub 2017 Oct 6 doi: 10.1007/s12306-017-0507-7. PMID: 28986742
Is intralesional resection suitable for central grade 1 chondrosarcoma: A systematic review and updated meta-analysis.
Chen X, Yu LJ, Peng HM, Jiang C, Ye CH, Zhu SB, Qian WW
Eur J Surg Oncol 2017 Sep;43(9):1718-1726. Epub 2017 Jun 14 doi: 10.1016/j.ejso.2017.05.022. PMID: 28666625
Laryngeal chondrosarcoma: A systematic review of 592 cases.
Chin OY, Dubal PM, Sheikh AB, Unsal AA, Park RC, Baredes S, Eloy JA
Laryngoscope 2017 Feb;127(2):430-439. Epub 2016 Jun 12 doi: 10.1002/lary.26068. PMID: 27291822

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