西山 悠也

OBJECTIVE: Central nervous system (CNS) solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms with a high propensity for local recurrence and extracranial metastasis. Although surgery and radiotherapy are the mainstays of treatment, systemic therapeutic options for recurrent disease remain limited. Pazopanib, a multitargeted tyrosine kinase inhibitor, has demonstrated clinical activity in extracranial SFTs; however, evidence in CNS SFTs is scarce. METHODS: We conducted a retrospective, single-institution study of patients with recurrent CNS SFTs treated with pazopanib. Clinical data, including prior treatments, imaging responses, treatment duration, and adverse events, were collected from medical records. Exploratory next-generation sequencing-based cancer panel testing was performed in two patients. RESULTS: Four patients with recurrent CNS SFTs were included. All had undergone prior surgical resection and radiotherapy. Pazopanib achieved partial response in one patient and stable disease in three patients, with treatment durations ranging from 7 months to over 2 years. One patient experienced disease progression after an initial period of response. Adverse events, including fatigue, gastrointestinal symptoms, and hypertension, were observed in all patients but were generally manageable with supportive care or dose adjustment. Exploratory molecular profiling identified various genomic alterations in two patients. CONCLUSIONS: In this single-institution retrospective series, pazopanib provided durable disease control with acceptable tolerability in selected patients with recurrent CNS SFTs. These findings support considering pazopanib as a systemic treatment option when further local therapies are not feasible, while highlighting the need for larger multicenter studies.

BACKGROUND: In >70% of patients with hemifacial spasm (HFS), the offending artery is either the anterior inferior cerebellar artery (AICA) or posterior inferior cerebellar artery (PICA), without a tortuous vertebrobasilar artery (VBA). We hypothesized that anchoring perforators around the root exit zone (REZ) of the AICA or PICA might induce vascular deviation and compression. We investigated the occurrence of these perforators from the AICA or PICA and the extent of VBA tortuosity to reveal the pathology of vascular compression. METHODS: This retrospective review included 110 patients after excluding those with vertebral artery (VA) compression alone. The occurrence of perforators was determined according to operative findings within 5 mm of the REZ, and VBA tortuosity was evaluated using MATLAB. We analyzed the association between perforators, VBA tortuosity, and the surgical implications. RESULTS: The occurrence of perforators from the offending AICA or PICA around the REZ was significantly higher in the group without VA compression (Group A) than in the group with VA compression (Group B). VBA tortuosity was significantly lower in Group A. VBA tortuosity was inversely correlated with the presence of AICA or PICA perforators in all 110 patients. Operative results were similar between the groups, although patients with low VBA tortuosity tended to require interposition in decompression procedures. CONCLUSIONS: Anchoring perforators around the REZ play a crucial role in vascular compression for patients with less tortuous VBAs. Moreover, surgeons should be prepared to deal with multiple perforators in a more complicated surgery in cases of less tortuous VBA.