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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

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
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

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
Low Grade Chondrosarcoma - Epidemiology, Diagnosis, Treatment.
Karpik M, Reszeć J
Ortop Traumatol Rehabil 2018 Feb 27;20(1):65-70. doi: 10.5604/01.3001.0011.5879. PMID: 30152763
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

Preclinical Characterization and Phase I Trial Results of INBRX-109, A Third-Generation, Recombinant, Humanized, Death Receptor 5 Agonist Antibody, in Chondrosarcoma.
Subbiah V, Chawla SP, Conley AP, Wilky BA, Tolcher A, Lakhani NJ, Berz D, Andrianov V, Crago W, Holcomb M, Hussain A, Veldstra C, Kalabus J, O'Neill B, Senne L, Rowell E, Heidt AB, Willis KM, Eckelman BP
Clin Cancer Res 2023 Aug 15;29(16):2988-3003. doi: 10.1158/1078-0432.CCR-23-0974. PMID: 37265425Free PMC Article
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

Preclinical Characterization and Phase I Trial Results of INBRX-109, A Third-Generation, Recombinant, Humanized, Death Receptor 5 Agonist Antibody, in Chondrosarcoma.
Subbiah V, Chawla SP, Conley AP, Wilky BA, Tolcher A, Lakhani NJ, Berz D, Andrianov V, Crago W, Holcomb M, Hussain A, Veldstra C, Kalabus J, O'Neill B, Senne L, Rowell E, Heidt AB, Willis KM, Eckelman BP
Clin Cancer Res 2023 Aug 15;29(16):2988-3003. doi: 10.1158/1078-0432.CCR-23-0974. PMID: 37265425Free 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
Extra-axial chordoma.
DiFrancesco LM, Davanzo Castillo CA, Temple WJ
Arch Pathol Lab Med 2006 Dec;130(12):1871-4. doi: 10.5858/2006-130-1871-EC. PMID: 17149966

Clinical prediction guides

Prognostic factors of sarcomas occurring in bone and joint: A SEER based study.
Zhou H, He S, Zhang D, Wang J, Yang X, Jiao J, Xu W, Yang J, Xiao J
Medicine (Baltimore) 2023 Oct 27;102(43):e34231. doi: 10.1097/MD.0000000000034231. PMID: 37904412Free PMC Article
SPECT/CT Radiomics for Differentiating between Enchondroma and Grade I Chondrosarcoma.
Yoon H, Choi WH, Joo MW, Ha S, Chung YA
Tomography 2023 Oct 16;9(5):1868-1875. doi: 10.3390/tomography9050148. PMID: 37888740Free PMC Article
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
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
Grading of bone tumors.
Unni KK, Dahlin DC
Semin Diagn Pathol 1984 Aug;1(3):165-72. PMID: 6400634

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
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
Chondrosarcomas of the hands and feet: A case series and systematic review of the literature.
Fayad LM, Ahlawat S, Khan MS, McCarthy E
Eur J Radiol 2015 Oct;84(10):2004-12. Epub 2015 Jul 2 doi: 10.1016/j.ejrad.2015.06.026. PMID: 26189572

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