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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
Clear cell chondrosarcoma of the larynx.
Hendriks T, Cardemil F, Sader C
BMJ Case Rep 2018 Sep 4;2018 doi: 10.1136/bcr-2018-226541. PMID: 30181407Free PMC Article
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
Low-grade chondrosarcoma of bone: experiences from the Vienna Bone and Soft Tissue Tumour Registry.
Funovics PT, Panotopoulos J, Sabeti-Aschraf M, Abdolvahab F, Funovics JM, Lang S, Kotz RI, Dominkus M
Int Orthop 2011 Jul;35(7):1049-56. Epub 2010 Aug 28 doi: 10.1007/s00264-010-1065-x. PMID: 20803012Free 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

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
How to Diagnose Enchondroma, Bone Infarct, and Chondrosarcoma.
Mulligan ME
Curr Probl Diagn Radiol 2019 May-Jun;48(3):262-273. Epub 2018 Apr 6 doi: 10.1067/j.cpradiol.2018.04.002. PMID: 29724496
Clear cell chondrosarcoma of the larynx.
Hendriks T, Cardemil F, Sader C
BMJ Case Rep 2018 Sep 4;2018 doi: 10.1136/bcr-2018-226541. PMID: 30181407Free 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
Low-grade cartilage tumors: diagnosis and treatment.
Ryzewicz M, Manaster BJ, Naar E, Lindeque B
Orthopedics 2007 Jan;30(1):35-46; quiz 47-8. doi: 10.3928/01477447-20070101-08. PMID: 17260660

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
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
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
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
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 of the larynx.
Rinaggio J, Duffey D, McGuff HS
Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004 Mar;97(3):369-75. doi: 10.1016/j.tripleo.2003.09.015. PMID: 15024362
Grading of bone tumors.
Unni KK, Dahlin DC
Semin Diagn Pathol 1984 Aug;1(3):165-72. PMID: 6400634

Clinical prediction guides

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
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
Radiofrequency ablation in the treatment of atypical cartilaginous tumours in the long bones: lessons learned from our experience.
Dierselhuis EF, Overbosch J, Kwee TC, Suurmeijer AJH, Ploegmakers JJW, Stevens M, Jutte PC
Skeletal Radiol 2019 Jun;48(6):881-887. Epub 2018 Sep 29 doi: 10.1007/s00256-018-3078-2. PMID: 30267104Free PMC Article
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
The utility of (18)F-FDG PET and PET/CT in the diagnosis and staging of chondrosarcoma: a meta-analysis.
Zhang Q, Xi Y, Li D, Yuan Z, Dong J
J Orthop Surg Res 2020 Jun 22;15(1):229. doi: 10.1186/s13018-020-01748-w. PMID: 32571371Free PMC Article
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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