Skip to main content

Advertisement

SpringerLink
Log in

Extra-skeletal intracranial mesenchymal chondrosarcoma: systematic-literature review

  • Review
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Background

Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection.

Methods

A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, “primary intracranial chondrosarcoma”, “extraskeletal mesenchymal chondrosarcoma”, “mesenchymal chondrosarcoma” and “pediatric”. Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described.

Results

The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2 years (range 2 months to 18 years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired.

Conclusion

IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis.  Safe Maximal resection followed by radiotherapy is the preferred treatment strategy.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

Data Availability

Data is provided within the manuscript.

References

  1. Beena D, Kattoor J, Mathews A (2021) Mesenchymal chondrosarcoma-a retrospective study. Gulf J Oncol 1(35):54–58

    Google Scholar 

  2. Bloch OG, Jian BJ, Yang I, Han SJ, Aranda D, Ahn BJ et al (2009) A systematic review of intracranial chondrosarcoma and survival. J Clin Neurosci 16(12):1547–1551

    Article  PubMed  PubMed Central  Google Scholar 

  3. Chen J-Y, Hsu S-S, Ho J-T (2004) Extraskeletal intracranial mesenchymal chondrosarcoma: case report and literature review. Kaohsiung J Med Sci 20(5):240–246

    Article  PubMed  Google Scholar 

  4. Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D et al (2021) The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol 23(8):1231–1251

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Lightenstein L, Bernstein D (1959) Unusual benign and malignant chondroid tumors of bone. A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors, including a few multicentric ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. Cancer 12:1142–1157

    Article  CAS  PubMed  Google Scholar 

  6. Dahlin DC, Henderson ED (1962) Mesenchymal chondrosarcoma. Further observations on a new entity. Cancer 15(2):410–417

    Article  CAS  PubMed  Google Scholar 

  7. Oruckaptan HH, Berker M, Soylemezoglu F, Ozcan OE (2001) Parafalcine chondrosarcoma: an unusual localization for a classical variant: case report and review of the literature. Surg Neurol 55(3):174–179

    Article  CAS  PubMed  Google Scholar 

  8. Aigner T, Loos S, Müller S, Sandell LJ, Unni KK, Kirchner T (2000) Cell differentiation and matrix gene expression in mesenchymal chondrosarcomas. Am J Pathol 156(4):1327–1335

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Shabani S, Kaushal M, Kaufman B, Knipstein J, Lawlor MW, Lew S et al (2019) Intracranial extraskeletal mesenchymal chondrosarcoma: case report and review of the literature of reported cases in adults and children. World Neurosurgery 129:302–310

    Article  PubMed  Google Scholar 

  10. Scheithauer BW, Rubinstein LJ (1978) Meningeal mesenchymal chondrosarcoma. Report of 8 cases with review of the literature. Cancer 42(6):2744–2752

    Article  CAS  PubMed  Google Scholar 

  11. Ostlere S, McDonald B, Athanasou N (1999) Mesenchymal chondrosarcoma associated with Goldenhar’s syndrome. Arch Orthop Trauma Surg 119:347–348

    Article  CAS  PubMed  Google Scholar 

  12. Ventura E, Dionísio S, Ferreira Â, Saleiro R, Marques H, Magalhães M et al (2020) Maxillary mesenchymal chondrosarcoma leading to a diagnosis of Li-Fraumeni syndrome. J Surg Case Rep 2020(1):rjz386

    Article  PubMed  PubMed Central  Google Scholar 

  13. Dini LI, Isolan GR, Saraiva GA, Dini SA, Gallo P (2007) Maffucci’s syndrome complicated by intracranial chondrosarcoma: Two new illustrative cases. Arq Neuropsiquiatr 65:816–821

    Article  PubMed  Google Scholar 

  14. Frezza AM, Cesari M, Baumhoer D, Biau D, Bielack S, Campanacci DA et al (2015) Mesenchymal chondrosarcoma: Prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study. Eur J Cancer 51(3):374–81

    Article  PubMed  Google Scholar 

  15. Chu J, Ma H, Wang Y, Li K, Liao C, Ding Y (2022) CT and MRI findings of intracranial extraskeletal mesenchymal chondrosarcoma—a case report and literature review. Transl Cancer Res 11(9):3409

    Article  PubMed  PubMed Central  Google Scholar 

  16. Sadashiva N, Sharma A, Shukla D, Rajalakshmi P, Mahadevan A, Devi BI (2016) Intracranial extraskeletal mesenchymal chondrosarcoma. World Neurosurg 95:618–e1

    Article  PubMed  Google Scholar 

  17. Chi J, Zhang M, Kang J (2016) Classical intracranial chondrosarcoma: a case report. Oncol Lett 12(5):4051–4053

    Article  PubMed  PubMed Central  Google Scholar 

  18. Yang MJ, Whelan R, Madden J, Mulcahy Levy JM, Kleinschmidt-DeMasters B, Hankinson TC et al (2018) Intracranial Ewing sarcoma: four pediatric examples. Childs Nerv Syst 34:441–448

    Article  PubMed  Google Scholar 

  19. Chen Q, Chen X-Z, Wang J-M, Li S-W, Jiang T, Dai J-P (2012) Intracranial meningeal hemangiopericytomas in children and adolescents: CT and MR imaging findings. Am J Neuroradiol 33(1):195–199

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Kumarasamy S, Garg K, Sharma MC, Chandra PS (2023) Intracranial myeloid sarcoma as the first presentation of acute myeloid leukemia and literature review. Childs Nerv Syst 39(12):3607–3612

    Article  PubMed  Google Scholar 

  21. Kumarasamy S, Satyarthee GD (2024) Supratentorial extra-ventricular ependymoma as a mass lesion in a child: report and literature review. Childs Nerv Syst 2:1–7

    Google Scholar 

  22. Choi JH, Ro JY (2021) The 2020 WHO classification of tumors of bone: an updated review. Adv Anat Pathol 28(3):119–138

    Article  CAS  PubMed  Google Scholar 

  23. Shakked RJ, Geller DS, Gorlick R, Dorfman HD (2012) Mesenchymal chondrosarcoma: Clinicopathologic study of 20 cases. Arch Pathol Lab Med 136(1):61–75

    Article  PubMed  Google Scholar 

  24. Arora K, Riddle ND (2018) Extraskeletal mesenchymal chondrosarcoma. Arch Pathol Lab Med 142(11):1421–1424

    Article  CAS  PubMed  Google Scholar 

  25. Yapıcıer Ö, Nanah AR, Taskapılıoglu MÖ, Demir MK (2021) Intracranial extra-axial mesenchymal chondrosarcoma in a 16-month-old patient with a literature review of pediatric patients. Childs Nerv Syst 37(2):649–657

    Article  PubMed  Google Scholar 

  26. El Beaino M, Roszik J, Livingston JA, Wang W-L, Lazar AJ, Amini B et al (2018) Mesenchymal chondrosarcoma: a review with emphasis on its fusion-driven biology. Curr Oncol Rep 20:1–11

    Article  Google Scholar 

  27. Neff B, Sataloff RT, Storey L, Hawkshaw M, Spiegel JR (2002) Chondrosarcoma of the skull base. Laryngoscope 112(1):134–139

    Article  PubMed  Google Scholar 

  28. Sikdar D, Gupta S, Roshan R, Agarwal S, Joseph DM, Gupta M (2023) Primary intracranial extraskeletal mesenchymal chondrosarcoma of the brain in a pediatric patient: A case report and review of literature. Indian J Med Paediat Oncol 14

  29. Isaacson B, Kutz JW, Roland PS (2007) Lesions of the petrous apex: diagnosis and management. Otolaryngol Clin North Am 40(3):479–519

    Article  PubMed  Google Scholar 

  30. Flyger G, Freidenfeldt H, Orell S (1963) Intracerebral, possibly malignant osteochondrofibroma in a child. Acta Pathol Microbiol Scand 58(3):299–305

    Article  CAS  PubMed  Google Scholar 

  31. Wu WQ, Lapi A (1970) Primary non-skeletal intracranial cartilaginous neoplasms: report of a chondroma and a mesenchymal chondrosarcoma. J Neurol Neurosurg Psychiatry 33(4):469–475

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Salvador AH, Beabout JW, Dahlin DC (1971) Mesenchymal chondrosarcoma—observations on 30 new cases. Cancer 28(3):605–615

    Article  CAS  PubMed  Google Scholar 

  33. Lynch P, Uriburu E (1973) An intracranial cartilage-containing meningeal tumor: case report. J Neurosurg 39(2):261–264

    Article  CAS  PubMed  Google Scholar 

  34. Rollo J, Green W, Kahn L (1979) Primary meningeal mesenchymal chondrosarcoma. Arch Pathol Lab Med 103(5):239–243

    CAS  PubMed  Google Scholar 

  35. Kobayashi T, Yoshida J, Kageyama N, Makita Y, Aoyama I, Yamashita J (1980) Mesenchymal chondrosarcoma arising from dura mater (author’s transl). No Shinkei Geka Neurol Surg 8(9):881–887

    CAS  Google Scholar 

  36. Rodda R, Franklin C (1984) Intracranial meningeal chondrosarcoma—probable mesenchymaltype. Aust N Z J Surg 54(4):387–390

    Article  CAS  PubMed  Google Scholar 

  37. Nagata S, Sawada K, Kitamura K (1986) Chondrosarcoma arising from the falx cerebri. Surg Neurol 25(5):505–509

    Article  CAS  PubMed  Google Scholar 

  38. Chhem R, Bui B, Calderon-Villar H, Fontaine S (1992) Case report: primary mesenchymal chondrosarcoma of the brain. Clin Radiol 45(6):422–423

    Article  CAS  PubMed  Google Scholar 

  39. Cho B-K, Chi JG, Wang KC, Chang KH, Choi KS (1993) Intracranial mesenchymal chondrosarcoma: a case report and literature review. Childs Nerv Syst 9:295–299

    Article  CAS  PubMed  Google Scholar 

  40. Schut L, Canady AI, Sutton LN, Bruce DA (1994) Meningeal tumors in children. Pediatr Neurosurg 20(3):207–213

    Article  CAS  PubMed  Google Scholar 

  41. Malik SN, Farmer PM, Hajdu SI, Rosenthal A (1996) Mesenchymal chondrosarcoma of the cerebellum. Ann Clin Lab Sci 26(6):496–500

    CAS  PubMed  Google Scholar 

  42. Rushing EJ, Armonda RA, Ansari Q, Mena H (1996) Mesenchymal chondrosarcoma: a clinicopathologic and flow cytometric study of 13 cases presenting in the central nervous system. Cancer: Interdiscip Int J Am Cancer Soc 77(9):1884–1891

    Article  CAS  Google Scholar 

  43. Nozaki K, Nagata I, Takahashi J, Hashimoto N (1999) Intracranial mesenchymal chondrosarcoma associated with a left transverse sigmoid dural AV fistule. Acta Neurochir 141(3):327

    Article  CAS  PubMed  Google Scholar 

  44. Crosswell H, Buchino JJ, Sweetman R, Reisner A (2000) Intracranial mesenchymal chondrosarcoma in an infant. Med Pediatr Oncol: Official J SIOP—Int Soc Pediatr Oncol (Soc Int Oncol Pédiatr) 34(5):370–374

    Article  CAS  Google Scholar 

  45. La Spina M, Dollo C, Giangaspero F, Bertolini P, Russo G (2003) Intracranial mesenchymal chondrosarcoma with osteoid formation: report of a pediatric case. Childs Nerv Syst 19:680–682

    Article  PubMed  Google Scholar 

  46. Huang Y-L, Lai P-H, Lin S-L, Yuan M-K, Pan H-B (2004) Mesenchymal chondrosarcoma at the falx cerebri. 中華放射線醫學雜誌 29(6):337–341

    Google Scholar 

  47. Waliuddin A, Jamjoom AB, Thomas J (2006) Intracranial extraskeletal mesenchymal chondrosarcoma. Neurosci J 11(3):205–209

    Google Scholar 

  48. De Cecio R, Migliaccio I, Falleti J, Del De Caro MB, Pettinato G (2008) Congenital intracranial mesenchymal chondrosarcoma: case report and review of the literature in pediatric patients. Pediatr Dev Pathol 11(4):309–313

    Article  PubMed  Google Scholar 

  49. Fanburg-Smith JC, Auerbach A, Marwaha JS, Wang Z, Santi M, Judkins AR et al (2010) Immunoprofile of mesenchymal chondrosarcoma: aberrant desmin and EMA expression, retention of INI1, and negative estrogen receptor in 22 female-predominant central nervous system and musculoskeletal cases. Ann Diagn Pathol 14(1):8–14

    Article  PubMed  Google Scholar 

  50. Sardi I, Massimino M, Genitori L, Buccoliero AM, Giangaspero F, Ferrari A (2011) Intracranial mesenchymal chondrosarcoma: report of two pediatric cases. Pediatr Blood Cancer 56(4):685–686

    Article  PubMed  Google Scholar 

  51. Fu L-Y, Han Q, Cheng P, Yang H-J, Zhao Y (2021) Rare case report and literature review of intracranial mesenchymal chondrosarcoma. Ann Palliat Med 10(11):12012–12017

    Article  PubMed  Google Scholar 

Download references

Funding

None.

Author information

Authors and Affiliations

  1. Department of Neurosurgery, All India Institute of Medical Sciences, Room No 720, CNC, New Delhi, India

    Sivaraman Kumarasamy, Kanwaljeet Garg, Manmohanjit Singh, Poodipedi Sarat Chandra & Shashank Sharad Kale

  2. Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India

    Ajay Garg

  3. Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India

    M. C. Sharma

Authors
  1. Sivaraman Kumarasamy
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kanwaljeet Garg
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ajay Garg
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M. C. Sharma
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Manmohanjit Singh
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Poodipedi Sarat Chandra
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Shashank Sharad Kale
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Sivaraman Kumarasamy — Conception & design of work, acquisition, analysis, interpretation of data and drafted the work. Kanwaljeet Garg — Analysis, interpretation of data and revised the work. Ajay Garg — Interpretation of data and revised the work. M C Sharma — Interpretation of data and revised the work. Manmohanjit singh — Interpretation of data and revised the work. Poodipedi Sarat Chandra — Interpretation of data and revised the work. Shashank Sharad Kale — Interpretation of data and revised the work. All authors have approved the submitted version (and any substantially modified version that involves the author’s contribution to the study) AND agreed both to be personally accountable for the contributions and to ensure that questions related to the accuracy or integrity of any part of the work.

Corresponding author

Correspondence to Kanwaljeet Garg.

Ethics declarations

Ethical approval

Consent to participate and consent for publication was taken from the patient using written informed consent to publish from the parent. Ethics committee approval was obtained.

Conflict of interest

The authors declare no competing interests

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kumarasamy, S., Garg, K., Garg, A. et al. Extra-skeletal intracranial mesenchymal chondrosarcoma: systematic-literature review. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06452-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s00381-024-06452-2

Keywords

Search

Navigation