Gross total resection is associated with improvement and prognosis even in von Hippel-Lindau disease-related hemangioblastomas: Nationwide registry in Japan.

Background: Although hemangioblastomas (HBs) are biologically benign, their management is often complicated, particularly in the context of von Hippel-Lindau disease (VHL). Few studies have investigated treatment outcomes of both VHL-related sporadic HBs in detail. This study assessed the clinical characteristics and neurosurgical outcomes of VHL-related and sporadic HBs using data from the nationwide Brain Tumor Registry of Japan database. Methods: Patients with HB who underwent surgery between 2001 and 2008 were included. Clinical and radiological findings, including preoperative and postoperative Karnofsky Performance Status (KPS) scores, were evaluated. Factors associated with improved postoperative KPS were identified using univariate and multivariate analyses. Postoperative outcomes were evaluated using the Kaplan-Meier method. Results: A total of 443 patients (68 with VHL) were analyzed, with a median follow-up duration of 57 months, and gross total resection (GTR) was achieved in 81% of patients. Compared to sporadic HB, VHL-related HB was associated with perioperative complications (P = .020), shorter recurrence-free survival (P < .001), and the formation of de novo lesions (P < .001). GTR significantly correlated with improved postoperative KPS (P = .002) after adjusting for disease etiology and overall survival (OS) (P < .001) in VHL-related HBs, as analyzed by the Kaplan-Meier method. Conclusions: The prognosis was worse for VHL-related HBs than for sporadic HBs; however, GTR was associated with improved OS, even in VHL-related HBs. Surgical resection is of the utmost importance in treating HB, regardless of the etiology.