Citations

The authors present the case of a man with a papillary craniopharyngioma, first diagnosed at 47 years of age, who experienced multiple recurrences. Review of the pathologic specimen from his first resection demonstrated the BRAF V600E mutation. With his most recent recurrence following previous surgery and radiotherapy, at 52 years of age, the decision was made to initiate treatment with the BRAF V600E inhibitor dabrafenib. Imaging following initiation of dabrafenib demonstrated reduction in tumor size. He remained on dabrafenib therapy for approximately 1 year and continued to demonstrate a good clinical result. At that time the decision was made to discontinue dabrafenib therapy and follow up with serial imaging. After more than 1 year of follow-up since stopping dabrafenib, the patient has continued to do well with no radiographic evidence of tumor progression and continues to be monitored with frequent interval imaging.

Abstract MEDICAL THERAPY Target Population Adults with histologically proven or suspected vestibular schwannomas with neurofibromatosis type 2 (NF2). Question What is the role of bevacizumab in the treatment of patients with vestibular schwannomas? Recommendations Level 3: It is recommended that bevacizumab be administered in order to radiographically reduce the size or prolong tumor stability in patients with NF2 without surgical options. Level 3: It is recommended that bevacizumab be administered to improve hearing or prolong time to hearing loss in patients with NF2 without surgical options. Question Is there a role for lapatinib, erlotinib, or everolimus in the treatment of patients with vestibular schwannomas? Recommendations Level 3: Lapatinib may be considered for use in reducing vestibular schwannoma size and improvement in hearing in NF2. Level 3: Erlotinib is not recommended for use in reducing vestibular schwannoma size or improvement in hearing in patients with NF2. Level 3: Everolimus is not recommended for use in reducing vestibular schwannoma size or improvement in hearing in NF2. Question What is the role of aspirin, to augment inflammatory response, in the treatment of patients with vestibular schwannomas? Target Population Any patient with a vestibular schwannoma undergoing observation. Recommendation Level 3: It is recommended that aspirin administration may be considered for use in patients undergoing observation of their vestibular schwannomas. Question Is there a role for treatment of vasospasm, ie, nimodipine or hydroxyethyl starch, perioperatively to improve facial nerve outcomes in patients with vestibular schwannomas? Target Population Adults with histologically proven or suspected vestibular schwannomas. Recommendation Level 3: Perioperative treatment with nimodipine (or with the addition of hydroxyethyl starch) should be considered to improve postoperative facial nerve outcomes and may improve hearing outcomes. PREHABILITATION Question Is there a role for preoperative vestibular rehab or vestibular ablation with gentamicin for patients surgically treated for vestibular schwannomas? Target Population Adults with histologically proven or suspected vestibular schwannomas. Recommendations Level 3: Preoperative vestibular rehabilitation is recommended to aid in postoperative mobility after vestibular schwannoma surgery. Level 3: Preoperative gentamicin ablation of the vestibular apparatus should be considered to improve postoperative mobility after vestibular schwannoma surgery. SURGICAL THERAPY Question Does endoscopic assistance make a difference in resection or outcomes in patients with vestibular schwannomas? Target Population Vestibular schwannoma patients, who are surgical candidates. Inclusion in this analysis required resection utilizing the endoscope, either as the primary operative visualization or microscopic assistance with more than 20 patients treated. Recommendation Level 3: Endoscopic assistance is a surgical technique that the surgeon may choose to use in order to aid in visualization. The full guideline can be found at: https://www.cns.org/guidelines/guidelines-management-patients-vestibular-schwannoma/chapter_9.

Abstract Background Despite advances in modern therapy, high-grade gliomas continue to portend a dismal prognosis and nearly all patients will experience relapse. Unfortunately, salvage options remain limited. In this study, we assessed outcomes for patients with recurrent gliomas treated with reirradiation. Methods We retrospectively identified 48 glioma patients treated with reirradiation between 2013 and 2016. All had radiographic or pathologic evidence of recurrence. Prognostic factors were abstracted from the electronic medical record. Results Initial surgery included biopsy in 15, subtotal resection in 21, and gross total resection in 12. Initial chemotherapy included temozolomide (TMZ) in 31, TMZ+dasatinib in 7, TMZ+vorinostat in 3, and procarbazine, lomustine, and vincristine in 2. The median dose of primary radiotherapy was 60 Gy delivered in 30 fractions. Median overall survival (OS) and progression-free survival (PFS) from initial diagnosis were 3.2 and 1.7 years, respectively. A total of 36 patients failed salvage bevacizumab before reirradiation. Salvage surgery was performed before reirradiation in 21 patients. Median time to reirradiation was 1.7 years. Median follow-up was 13.7 months from reirradiation. Concurrent systemic therapy was given in 33 patients (bevacizumab in 27, TMZ in 8, and lomustine in 2). Median PFS and OS after reirradiation were 3.2 and 6.3 months, respectively. Radionecrosis occurred in 4 patients and no radionecrosis was seen in patients receiving concurrent bevacizumab with reirradiation (0% vs 19%, P = .03). Conclusions Reirradiation may result in delayed tumor progression with acceptable toxicity. Prospective trials are needed to determine the impact of reirradiation on tumor progression and quality of life.