Skull base chordoma treated with proton therapy: A systematic review

Background: Chordoma located in the skull base is usually a challenging surgical condition. It is often not possible to achieve gross total resection. Residual tumors have been treated with adjuvant focal radiation therapy employing high-energy particles most commonly through proton beam. In this review, we systematically analyzed indications and outcomes of this treatment with respect to local control rates of the lesion and factors determining recurrence of skull base chordomas. In addition, we collected data on treatment-associated radiation-induced side effects.

Methods: In line with the PRISMA guidelines, the authors performed a literature search algorithm for relevant articles using three databases: PubMed, Embase, and Cochrane. Inclusion and exclusion criteria were applied to evaluate all identified studies published between 1980 and 2018.

Results: Our review included 11 studies for analysis (n = 511 patients). The mean age of the study population was 47.3 ± 5.8 years. The mean dose of postsurgical irradiation at the time of initial treatment was 71.1 ± 3.1 Gy. The mean follow-up duration was 45.0 ± 17.5 months. Within this follow-up duration, recurrence occurred in 26.8% of the patients. The mean time to recurrence was 34.5 ± 15.2 months. A significant number of patients experienced side effects varying from Grade 1 (mild dermatitis) to Grade 4 (temporal lobe necrosis and visual disorders).

Conclusion: Despite advances in proton therapy, recurrence rates in skull base chordoma remain high. The toxicity of proton therapy may be more prevalent than generally thought. Unfortunately, there is substantial variation in the methods of data reporting.