Chondrosarcoma
Last edit by Alaric Steinmetz on
A chondrosarcoma is a malignant bone tumor whose cells produce cartilage matrix but, unlike osteosarcoma, do not produce bone substance (osteoid). Unlike chondroma, chondrosarcoma exhibits greater cellularity, pleomorphism, and nuclear atypia.
Epidemiology
Intracranial chondrosarcomas are very rare tumors and account for approximately 0.15% of all intracranial tumors1.
Associated Diseases
The majority of intracranial chondrosarcomas are sporadic. Chondrosarcomas may occur more frequently in the following diseases2:
- Ollier disease
- Maffucci syndrome
- Paget disease
- Osteochondromas
WHO Classification
According to the WHO classification, chondrosarcomas are classified as Grade I–IV. In a case series of 200 patients with intracranial chondrosarcomas, 51% were Grade I, 21% were Grade II, and 29% showed a mixture of Grade I & I II tumors. No Grade III or Grade IV chondrosarcomas were reported in this case series3 4.
Imaging
Location
Skull base chondrosarcomas arise from the petroclival fissure in 66% of cases, from the clivus in 28%, and from the sphenoethmoidal complex in 6%5.
Prognosis
The 5-year mortality rate for intracranial chondrosarcomas is approximately 11.5%6. The overall survival rate, independent of WHO grade, is 75-100% at 5 years and 71-100% at 10 years7 8 9 10.
References
Cianfriglia, F., Pompili, A. & Occhipinti, E. Intracranial malignant cartilaginous tumours. Report of two cases and review of literature. Acta neurochir 45, 163–175 (1978). ↩ ↩
Neurosurgery Case Review: Questions and Answers. Remi Nader, Abdulrahman J Sabbagh. 2020 Thieme Verlage. ISBN 9781626231986 ↩ ↩
Rosenberg, Andrew E., et al. "Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma." The American journal of surgical pathology 23.11 (1999): 1370. ↩ ↩ ↩ ↩
Koch, Brenton B., et al. "National cancer database report on chondrosarcoma of the head and neck." Head & Neck: Journal for the Sciences and Specialties of the Head and Neck 22.4 (2000): 408-425. ↩ ↩
Bloch, Orin G., et al. "A systematic review of intracranial chondrosarcoma and survival." Journal of Clinical Neuroscience 16.12 (2009): 1547-1551. ↩ ↩
Oghalai, John S., et al. "Skull base chondrosarcoma originating from the petroclival junction." Otology & Neurotology 26.5 (2005): 1052-1060. ↩ ↩
Carlson, Matthew L., et al. "Petroclival chondrosarcoma: a multicenter review of 55 cases and new staging system." Otology & Neurotology 37.7 (2016): 940-950. ↩ ↩
Uhl, Matthias, et al. "High control rate in patients with chondrosarcoma of the skull base after carbon ion therapy: first report of long‐term results." Cancer 120.10 (2014): 1579-1585. ↩ ↩
Wanebo, John E., et al. "Management of cranial base chondrosarcomas." Neurosurgery 58.2 (2006): 249-255. ↩ ↩
