The two faces of hemangioblastoma: Examining the differences between sporadic and familial hemangioblastomas

Abstract Purpose: Intracranial hemangioblastomas are rare tumors that present operative challenges due to their vascularity. The incidence of hemangioblastomas increases to 60%–80% in patients with von Hippel–Lindau (VHL) disease. We sought to compare clinical management strategies between VHL-related (VHL) and sporadic hemangioblastomas (SH). Materials and Methods: We conducted a retrospective review of all intracranial hemangioblastomas resected from 2008 to 2023 at our institution. We collected patient data on patients with pathology-confirmed intracranial hemangioblastomas. Results: We identified 56 hemangioblastoma resections in 48 patients, and 42.9% of patients had VHL. SH patients primarily presented with headache (71.0%), and significantly more SHs presented with headache than VHs (26.6%) ( P = 0.001). Other than preoperative hemoglobin (14.7 ± 3.47 in SH versus 13.3 ± 4.54 in VH) ( P = 0.025), tumor characteristics did not significantly differ between groups. Preoperative embolization was performed at a similar rate in both groups ( P = 0.642), while preoperative radiation was significantly more common for VHL (41.7%) than SH (0%) ( P < 0.001). A suboccipital surgical approach was performed on both groups (SH 93.8%, VHL 87.5%). Estimated blood loss was similar for SH and VHL patients ( P = 0.992); however, postoperative hemoglobin was significantly less among VHL (11.8 ± 4.36) than SH (13.0 ± 3.05) ( P = 0.047) patients. Postoperative complications, tumor recurrence, and time to recurrence did not significantly differ between groups, although the association between VHL status and neurologic deficits approached significance ( P = 0.096). Conclusion: Although the VHL mutation in VHL may theoretically impact blood loss and patient outcomes during surgical management of hemangioblastomas, we found both SH and VHL patients to have relatively similar tumor presentations and postoperative courses.