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Holtzman et al., reviewed the medical records of 43 patients with a median age of 49 years (range, 23-80 years) treated with double-scattered 3D conformal proton therapy for skull-base chondrosarcomas between January 2007 and February 2016. Proton therapy-related toxicities were scored using CTCAE v4.0.

The median radiotherapy dose was 73.8 Gy(RBE) (range, 64.5-74.4 Gy[RBE]). Thirty-six (84%) and 7 (16%) patients underwent surgical resection or biopsy alone. Tumor grade distribution included: grade 1, 19 (44%) patients; grade 2, 22 (51%); and grade 3, 2 (5%). Forty patients had gross disease at the time of radiotherapy and 7 patients were treated for locally recurrent disease following surgery. The median follow-up was 3.7 years (range, 0.7-10.1 years). There were no acute grade 3 toxicities related to RT. At 4 years following RT, actuarial rates of overall survival, cause-specific survival, local control, and RT-related grade 3 toxicity-free survival were 95%, 100%, 89%, and 95%.

High-dose, double-scattered 3D conformal proton therapy alone or following surgical resection for skull-base chondrosarcoma is an effective treatment with a high rate of local control with no acute grade 3 radiation-related toxicity. Further follow-up of this cohort is necessary to better characterize long-term disease control and late toxicities 1).


The potential malignant progression of enchondroma into a secondary chondrosarcoma is a well-known fact. Nevertheless, chondrosarcoma located at the skull base in patients with Maffuci syndrome is a very rare condition, with only 18 cases reported in the literature.

Beer-Furlan et al. report 2 other cases successfully treated through an expanded endoscopic endonasal approach and discuss the condition based on the literature review.

Skull base chondrosarcoma associated with Maffucci syndrome is a rare condition. The disease cannot be cured, therefore surgical treatment should be performed in symptomatic patients aiming for maximal tumor resection with function preservation. The endoscopic endonasal approach is a safe and reliable alternative for the management of these tumors 2).

Holtzman AL, Rotondo RL, Rutenberg MS, Indelicato DJ, Mercado CE, Rao D, Tavanaiepour D, Morris CG, Louis D, Flampouri S, Mendenhall WM. Proton therapy for skull-base chondrosarcoma, a single-institution outcomes study. J Neurooncol. 2019 Mar 2. doi: 10.1007/s11060-019-03129-8. [Epub ahead of print] PubMed PMID: 30827010.
Beer-Furlan A, Balsalobre L, Vellutini EA, Stamm AC. Endoscopic Endonasal Approach in Skull Base Chondrosarcoma Associated with Maffucci Syndrome: Case Series and Literature Review. World Neurosurg. 2016 Jan;85:365.e7-15. doi: 10.1016/j.wneu.2015.08.070. Epub 2015 Sep 5. Review. PubMed PMID: 26348567.
chondrosarcoma_case_series.txt · Last modified: 2019/07/12 15:10 by administrador