Diagnostic and Management Challenges in Chondrosarcoma: A Case-Based Expert Review
Abstract
This case-based expert review from ORTHOBIT 2025 Congress (Tehran, Iran) highlights the practical challenge of distinguishing between enchondroma/atypical cartilaginous tumor (ACT) (low-grade) and higher-grade chondrosarcoma (CS). A 57-year-old woman presented with 4 months of shoulder pain. Magnetic resonance imaging (MRI) revealed a proximal humeral metaphyseal intramedullary cartilaginous lesion with a focal cortical breach and a small extraosseous component. The core needle biopsy (CNB) suggested enchondroma, and the patient underwent extended curettage, cementation, and plate fixation. Final pathology upgraded the lesion to central CS, grade 2, prompting resection and reconstruction using a proximal humeral megaprosthesis. At 3 years, she was pain-free, recurrence-free, The Musculoskeletal Tumor Society Score (MSTS) 26/30, with moderate range of motion (ROM) limitation, and radiographic superior prosthetic migration without major complications. This case emphasizes the importance of radiology-pathology correlation and planning for possible diagnostic upgrading in borderline cartilaginous tumors.
1. Gazendam A, Popovic S, Parasu N, Ghert M. Chondrosarcoma: A Clinical Review. J Clin Med. 2023;12(7):2506. doi: 10.3390/jcm12072506. [PubMed: 37048590]. [PubMed Central: PMC10095313].
2. Anderson WJ, Doyle LA. Updates from the 2020 World Health Organization Classification of Soft Tissue and Bone Tumours. Histopathology. 2021;78(5):644-57. doi: 10.1111/his.14265. [PubMed: 33438273].
3. Rosenberg AE. WHO Classification of Soft Tissue and Bone, fourth edition: summary and commentary. Curr Opin Oncol. 2013;25(5):571-3. doi: 10.1097/01.cco.0000432522.16734.2d. [PubMed: 23942303].
4. Gomez-Pena S, Rueda de Eusebio Á, Arrazola García J, Romero Fernández P, Moreno Casado MJ, Crespo Rodríguez AM. Update of cartilaginous tumours according to the WHO classification 2020. Radiologia (Engl Ed). 2024;66(1):57-69. doi: 10.1016/j.rxeng.2023.05.006. [PubMed: 38365355].
5. Van Praag Veroniek VM, Rueten-Budde AJ, Ho V, Dijkstra PDS, Fiocco M, Van De Sande MAJ. Incidence, outcomes and prognostic factors during 25 years of treatment of chondrosarcomas. Surg Oncol. 2018;27(3):402-8. doi: 10.1016/j.suronc.2018.05.009. [PubMed: 30217294].
6. Jeys LM, Thorkildsen J, Kurisunkal V, Puri A, Ruggieri P, Houdek MT, et al. Controversies in orthopaedic oncology. Bone Joint J. 2024;106-b(5):425-9. doi: 10.1302/0301-620x.106b5.Bjj-2023-1381. [PubMed: 38689572].
7. Ebrahimpour A, Chehrassan M, Karimi A, Sabaghzadeh A, Kafiabadi MJ, Biglari F, et al. The Epidemiology of Chondrosarcoma in Iran Based on Iran National Cancer Registry. Int J Cancer Manag. 2022;15(10):e119308. doi: 10.5812/ijcm-119308.
8. Savci-Heijink C, Cleven A, Bovee J. Benign and low-grade cartilaginous tumors: an update on differential diagnosis. Diagn Histopathol 2022;28(12):501-9. doi: 10.1016/j.mpdhp.2022.09.001.
9. Wells ME, Eckhoff MD, Kafchinski LA, Polfer EM, Potter BK. Conventional Cartilaginous Tumors: Evaluation and Treatment. JBJS Rev. 2021;9(5):e20. doi: 10.2106/jbjs.Rvw.20.00159. [PubMed: 34881859].
10. Saoud C, Lam H, Gross JM, Ali SZ. Fine needle aspiration of bone lesions: A tertiary care centre experience. Cytopathology. 2023;34(6):562-72. doi: 10.1111/cyt.13270. [PubMed: 37434498].
11. Boudou-Rouquette P, Corradini N, Sunyach M-P, Larousserie F, Cordero C, Cardine AM, et al. Chondrosarcomas: Multidisciplinary review and practical recommendations, on behalf of GroupOs. Bull Cancer. 2025;112(6):638-55. doi: 10.1016/j.bulcan.2024.07.013.
12. Carter JM, Inwards CY. Conventional chondrosarcoma: old controversies and new insights. Diagn Histopathol. 2014;20(5): 181-9. doi: 10.1016/j.mpdhp.2014.03.004.
13. Hodel S, Laux C, Farei-Campagna J, Götschi T, Bode-Lesniewska B, Müller DA. The impact of biopsy sampling errors and the quality of surgical margins on local recurrence and survival in chondrosarcoma. Cancer Manag Res. 2018;10:3765-71. doi: 10.2147/cmar.S178768. [PubMed: 30288107]. [PubMed Central: PMC6159809].
14. Jennings R, Riley N, Rose B, Rossi R, Skinner JA, Cannon SR, et al. An evaluation of the diagnostic accuracy of the grade of preoperative biopsy compared to surgical excision in chondrosarcoma of the long bones. Int J Surg Oncol. 2010;2010:270195. doi: 10.1155/2010/270195. [PubMed: 22312488]. [PubMed Central: PMC3265259].
15. Jo I, Gould D, Schlicht S, Taubman K, Choong P. Diagnostic accuracy of functional imaging modalities for chondrosarcoma: A systematic review and meta-analysis. J Bone Oncol. 2019;19:100262. doi: 10.1016/j.jbo.2019.100262. [PubMed: 31667063]. [PubMed Central: PMC6811998].
16. Saberi S, Bagheri N, Kalantar SH, Khabiri SS. Orthopedic oncology residency education, how broad and how deep? J Adv Med Educ Prof. 2023;11(4):262-4. https://doi.org/10.30476/jamp.2023.98678.1804. doi: [PubMed: 37901756]. [PubMed Central: PMC10611936].
17. Saberi S, Bagheri N, Kalantar SH, Saffar H, Khabiri SS. Gorham Stout syndrome, the challenge in diagnosis and unique in treatment: a case report. J Med Case Rep. 2023;17(1):360. doi: 10.1186/s13256-023-04094-7. [PubMed: 37605280]. [PubMed Central: PMC10463799].
18. Eefting D, Schrage YM, Geirnaerdt MJ, Le Cessie S, Taminiau AH, Bovée JV, et al. Assessment of interobserver variability and histologic parameters to improve reliability in classification and grading of central cartilaginous tumors. Am J Surg Pathol. 2009;33(1):50-7. doi: 10.1097/PAS.0b013e31817eec2b. [PubMed: 18852676].
19. Crim J, Schmidt R, Layfield L, Hanrahan C, Manaster BJ. Can imaging criteria distinguish enchondroma from grade 1 chondrosarcoma? Eur J Radiol. 2015;84(11):2222-30. doi: 10.1016/j.ejrad.2015.06.033. [PubMed: 26220916].
20. Weinschenk RC, Wang WL, Lewis VO. Chondrosarcoma. J Am Acad Orthop Surg. 2021;29(13):553-62. doi: 10.5435/jaaos-d-20 01188. [PubMed: 33595238].
21. Afonso PD, Isaac A, Villagrán JM. Chondroid Tumors as Incidental Findings and Differential Diagnosis between Enchondromas and Low-grade Chondrosarcomas. Semin Musculoskelet Radiol. 2019;23(1):3-18. doi: 10.1055/s-0038-1675550. [PubMed: 30699449].
22. Douis H, Parry M, Vaiyapuri S, Davies AM. What are the differentiating clinical and MRI-features of enchondromas from low-grade chondrosarcomas? Eur Radiol. 2018;28(1): 398-409. doi: 10.1007/s00330-017-4947-0. [PubMed: 28695356].
23. Alhumaid SM, Alharbi At, Aljubair H. Magnetic Resonance Imaging Role in the Differentiation Between Atypical Cartilaginous Tumors and High-Grade Chondrosarcoma: An Updated Systematic Review. Cureus. 2020;12(10):e11237. doi: 10.7759/cureus.11237. [PubMed: 33269165]. [PubMed Central: PMC7704161].
24. Landuzzi L, Ruzzi F, Lollini PL, Scotlandi K. Chondrosarcoma: New Molecular Insights, Challenges in Near-Patient Preclinical Modeling, and Therapeutic Approaches. Int J Mol Sci. 2025;26(4):1542. doi: 10.3390/ijms26041542. 40004005]. [PubMed Central: PMC11855192]. [PubMed:
25. Chen X, Yu LJ, Peng HM, Jiang C, Ye CH, Zhu SB, et al. Is intralesional resection suitable for central grade 1 chondrosarcoma: A systematic review and updated meta analysis. Eur J Surg Oncol. 2017;43(9):1718-26. doi: 10.1016/j.ejso.2017.05.022. [PubMed: 28666625].
26. Lone SN, Nisar S, Masoodi T, Singh M, Rizwan A, Hashem S, et al. Liquid biopsy: a step closer to transform diagnosis, prognosis and future of cancer treatments. Mol Cancer. 2022;21(1):79. doi: 10.1186/s12943-022-01543-7. 35303879]. [PubMed Central: PMC8932066]. [PubMed:
27. Aran V, Devalle S, Meohas W, Heringer M, Cunha Caruso A, Pinheiro Aguiar D, et al. Osteosarcoma, chondrosarcoma and Ewing sarcoma: Clinical aspects, biomarker discovery and liquid biopsy. Crit Rev Oncol Hematol. 2021;162:103340. doi: 10.1016/j.critrevonc.2021.103340. [PubMed: 33894338].
28. Lyskjaer I, Davies C, Strobl AC, Hindley J, James S, Lalam RK, et al. Circulating tumour DNA is a promising biomarker for risk stratification of central chondrosarcoma with IDH1/2 and GNAS mutations. Mol Oncol. 2021;15(12):3679-90. doi: 10.1002/1878-0261.13102. [PubMed: 34528398]. [PubMed Central: PMC8637565].
29. Gutteridge A, Rathbone VM, Gibbons R, Bi M, Archard N, Davies KEJ, et al. Digital PCR analysis of circulating tumor DNA: a biomarker for chondrosarcoma diagnosis, prognostication, and residual disease detection. Cancer Med. 2017;6(10): 2194-202. doi: 10.1002/cam4.1146. [PubMed: 28834325]. [PubMed Central: PMC5633548].
30. Wagner MJ, Pimenta EM, Sweeney NW, Loggers ET, Roberts JL, Brinkman E, et al. Genomic Characterization of Chondrosarcoma Reveals Potential Therapeutic Targets. JCO Precis Oncol. 2025;9:e2400592. doi: 10.1200/po-24-00592. [PubMed: 40117529]. [PubMed Central: PMC11949235].
2. Anderson WJ, Doyle LA. Updates from the 2020 World Health Organization Classification of Soft Tissue and Bone Tumours. Histopathology. 2021;78(5):644-57. doi: 10.1111/his.14265. [PubMed: 33438273].
3. Rosenberg AE. WHO Classification of Soft Tissue and Bone, fourth edition: summary and commentary. Curr Opin Oncol. 2013;25(5):571-3. doi: 10.1097/01.cco.0000432522.16734.2d. [PubMed: 23942303].
4. Gomez-Pena S, Rueda de Eusebio Á, Arrazola García J, Romero Fernández P, Moreno Casado MJ, Crespo Rodríguez AM. Update of cartilaginous tumours according to the WHO classification 2020. Radiologia (Engl Ed). 2024;66(1):57-69. doi: 10.1016/j.rxeng.2023.05.006. [PubMed: 38365355].
5. Van Praag Veroniek VM, Rueten-Budde AJ, Ho V, Dijkstra PDS, Fiocco M, Van De Sande MAJ. Incidence, outcomes and prognostic factors during 25 years of treatment of chondrosarcomas. Surg Oncol. 2018;27(3):402-8. doi: 10.1016/j.suronc.2018.05.009. [PubMed: 30217294].
6. Jeys LM, Thorkildsen J, Kurisunkal V, Puri A, Ruggieri P, Houdek MT, et al. Controversies in orthopaedic oncology. Bone Joint J. 2024;106-b(5):425-9. doi: 10.1302/0301-620x.106b5.Bjj-2023-1381. [PubMed: 38689572].
7. Ebrahimpour A, Chehrassan M, Karimi A, Sabaghzadeh A, Kafiabadi MJ, Biglari F, et al. The Epidemiology of Chondrosarcoma in Iran Based on Iran National Cancer Registry. Int J Cancer Manag. 2022;15(10):e119308. doi: 10.5812/ijcm-119308.
8. Savci-Heijink C, Cleven A, Bovee J. Benign and low-grade cartilaginous tumors: an update on differential diagnosis. Diagn Histopathol 2022;28(12):501-9. doi: 10.1016/j.mpdhp.2022.09.001.
9. Wells ME, Eckhoff MD, Kafchinski LA, Polfer EM, Potter BK. Conventional Cartilaginous Tumors: Evaluation and Treatment. JBJS Rev. 2021;9(5):e20. doi: 10.2106/jbjs.Rvw.20.00159. [PubMed: 34881859].
10. Saoud C, Lam H, Gross JM, Ali SZ. Fine needle aspiration of bone lesions: A tertiary care centre experience. Cytopathology. 2023;34(6):562-72. doi: 10.1111/cyt.13270. [PubMed: 37434498].
11. Boudou-Rouquette P, Corradini N, Sunyach M-P, Larousserie F, Cordero C, Cardine AM, et al. Chondrosarcomas: Multidisciplinary review and practical recommendations, on behalf of GroupOs. Bull Cancer. 2025;112(6):638-55. doi: 10.1016/j.bulcan.2024.07.013.
12. Carter JM, Inwards CY. Conventional chondrosarcoma: old controversies and new insights. Diagn Histopathol. 2014;20(5): 181-9. doi: 10.1016/j.mpdhp.2014.03.004.
13. Hodel S, Laux C, Farei-Campagna J, Götschi T, Bode-Lesniewska B, Müller DA. The impact of biopsy sampling errors and the quality of surgical margins on local recurrence and survival in chondrosarcoma. Cancer Manag Res. 2018;10:3765-71. doi: 10.2147/cmar.S178768. [PubMed: 30288107]. [PubMed Central: PMC6159809].
14. Jennings R, Riley N, Rose B, Rossi R, Skinner JA, Cannon SR, et al. An evaluation of the diagnostic accuracy of the grade of preoperative biopsy compared to surgical excision in chondrosarcoma of the long bones. Int J Surg Oncol. 2010;2010:270195. doi: 10.1155/2010/270195. [PubMed: 22312488]. [PubMed Central: PMC3265259].
15. Jo I, Gould D, Schlicht S, Taubman K, Choong P. Diagnostic accuracy of functional imaging modalities for chondrosarcoma: A systematic review and meta-analysis. J Bone Oncol. 2019;19:100262. doi: 10.1016/j.jbo.2019.100262. [PubMed: 31667063]. [PubMed Central: PMC6811998].
16. Saberi S, Bagheri N, Kalantar SH, Khabiri SS. Orthopedic oncology residency education, how broad and how deep? J Adv Med Educ Prof. 2023;11(4):262-4. https://doi.org/10.30476/jamp.2023.98678.1804. doi: [PubMed: 37901756]. [PubMed Central: PMC10611936].
17. Saberi S, Bagheri N, Kalantar SH, Saffar H, Khabiri SS. Gorham Stout syndrome, the challenge in diagnosis and unique in treatment: a case report. J Med Case Rep. 2023;17(1):360. doi: 10.1186/s13256-023-04094-7. [PubMed: 37605280]. [PubMed Central: PMC10463799].
18. Eefting D, Schrage YM, Geirnaerdt MJ, Le Cessie S, Taminiau AH, Bovée JV, et al. Assessment of interobserver variability and histologic parameters to improve reliability in classification and grading of central cartilaginous tumors. Am J Surg Pathol. 2009;33(1):50-7. doi: 10.1097/PAS.0b013e31817eec2b. [PubMed: 18852676].
19. Crim J, Schmidt R, Layfield L, Hanrahan C, Manaster BJ. Can imaging criteria distinguish enchondroma from grade 1 chondrosarcoma? Eur J Radiol. 2015;84(11):2222-30. doi: 10.1016/j.ejrad.2015.06.033. [PubMed: 26220916].
20. Weinschenk RC, Wang WL, Lewis VO. Chondrosarcoma. J Am Acad Orthop Surg. 2021;29(13):553-62. doi: 10.5435/jaaos-d-20 01188. [PubMed: 33595238].
21. Afonso PD, Isaac A, Villagrán JM. Chondroid Tumors as Incidental Findings and Differential Diagnosis between Enchondromas and Low-grade Chondrosarcomas. Semin Musculoskelet Radiol. 2019;23(1):3-18. doi: 10.1055/s-0038-1675550. [PubMed: 30699449].
22. Douis H, Parry M, Vaiyapuri S, Davies AM. What are the differentiating clinical and MRI-features of enchondromas from low-grade chondrosarcomas? Eur Radiol. 2018;28(1): 398-409. doi: 10.1007/s00330-017-4947-0. [PubMed: 28695356].
23. Alhumaid SM, Alharbi At, Aljubair H. Magnetic Resonance Imaging Role in the Differentiation Between Atypical Cartilaginous Tumors and High-Grade Chondrosarcoma: An Updated Systematic Review. Cureus. 2020;12(10):e11237. doi: 10.7759/cureus.11237. [PubMed: 33269165]. [PubMed Central: PMC7704161].
24. Landuzzi L, Ruzzi F, Lollini PL, Scotlandi K. Chondrosarcoma: New Molecular Insights, Challenges in Near-Patient Preclinical Modeling, and Therapeutic Approaches. Int J Mol Sci. 2025;26(4):1542. doi: 10.3390/ijms26041542. 40004005]. [PubMed Central: PMC11855192]. [PubMed:
25. Chen X, Yu LJ, Peng HM, Jiang C, Ye CH, Zhu SB, et al. Is intralesional resection suitable for central grade 1 chondrosarcoma: A systematic review and updated meta analysis. Eur J Surg Oncol. 2017;43(9):1718-26. doi: 10.1016/j.ejso.2017.05.022. [PubMed: 28666625].
26. Lone SN, Nisar S, Masoodi T, Singh M, Rizwan A, Hashem S, et al. Liquid biopsy: a step closer to transform diagnosis, prognosis and future of cancer treatments. Mol Cancer. 2022;21(1):79. doi: 10.1186/s12943-022-01543-7. 35303879]. [PubMed Central: PMC8932066]. [PubMed:
27. Aran V, Devalle S, Meohas W, Heringer M, Cunha Caruso A, Pinheiro Aguiar D, et al. Osteosarcoma, chondrosarcoma and Ewing sarcoma: Clinical aspects, biomarker discovery and liquid biopsy. Crit Rev Oncol Hematol. 2021;162:103340. doi: 10.1016/j.critrevonc.2021.103340. [PubMed: 33894338].
28. Lyskjaer I, Davies C, Strobl AC, Hindley J, James S, Lalam RK, et al. Circulating tumour DNA is a promising biomarker for risk stratification of central chondrosarcoma with IDH1/2 and GNAS mutations. Mol Oncol. 2021;15(12):3679-90. doi: 10.1002/1878-0261.13102. [PubMed: 34528398]. [PubMed Central: PMC8637565].
29. Gutteridge A, Rathbone VM, Gibbons R, Bi M, Archard N, Davies KEJ, et al. Digital PCR analysis of circulating tumor DNA: a biomarker for chondrosarcoma diagnosis, prognostication, and residual disease detection. Cancer Med. 2017;6(10): 2194-202. doi: 10.1002/cam4.1146. [PubMed: 28834325]. [PubMed Central: PMC5633548].
30. Wagner MJ, Pimenta EM, Sweeney NW, Loggers ET, Roberts JL, Brinkman E, et al. Genomic Characterization of Chondrosarcoma Reveals Potential Therapeutic Targets. JCO Precis Oncol. 2025;9:e2400592. doi: 10.1200/po-24-00592. [PubMed: 40117529]. [PubMed Central: PMC11949235].
| Files | ||
| Issue | Vol 12 No 2 (2026) | |
| Section | Case Report | |
| DOI | https://doi.org/10.18502/jost.v12i2.21517 | |
| Keywords | ||
| Chondrosarcoma Bone Neoplasms Magnetic Resonance Imaging Biopsy | ||
| Rights and permissions | |
|
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Saberi S, Valinejad-Qanati A, Khabiri SS. Diagnostic and Management Challenges in Chondrosarcoma: A Case-Based Expert Review. J Orthop Spine Trauma. 2026;12(2):107-11.
