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.
Vestibular schwannoma (VS) is a benign primary brain tumor that occurs sporadic or as part of a genetic syndrome. The most common cause is the mutation of the NF2 tumor suppressor gene that is involved in the production of the protein merlin. Merlin plays a role in cell growth and cell adhesion. In patients with NF2, the VSs arise bilaterally and coincide with other brain tumors. In sporadic VS, the tumor is typically unilateral and does not coincide in combination with other tumors. MRI is the standard imaging technique and can be used to assess the size and aspect of the tumor as well as the progression of disease. The preferred management of large VS in both VS types is surgery with or without adjuvant radiation. The management for the medium- or small-sized VS includes wait and scan, radiotherapy and/or surgery. This choice depends on the preference of the patient and institutional protocols. The outcomes of surgical and radiotherapy treatments are improving due to progress in surgical equipment/approaches, advances in radiation delivery techniques and dose optimizations protocols. The main purpose of the management of VS is preserving function as long as possible in combination with tumor control.
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