Sporadic and von Hippel–Lindau Related Hemangioblastomas of Brain and Spinal Cord: Multimodal Imaging for Intraoperative Strategy

SIMPLE SUMMARY: Central Nervous System (CNS) hemangioblastomas (HBs) are rare and benign tumors that may be sporadic or hereditary, linked mainly to von Hippel–Lindau disease. Considering their slow-growing behavior and the possible risks related to surgical treatment, a careful, tailored approach should be adopted. This work aims to provide a systematic overview of the clinical appearance and neuroradiological characteristics of CNS HBs, along with a report of clinical–radiological data of a mono-institutional retrospective cohort, with particular emphasis on the possible role of intraoperative multimodal imaging to guide surgical strategy. ABSTRACT: Hemangioblastomas (HBs) are rare, benign tumors often related to von Hippel–Lindau disease. They represent the most frequent primary cerebellar tumors in adults. Neurosurgical procedures aim to obtain a gross-total resection of tumor nodules, avoiding intra-postoperative hemorrhage. The introduction of new intraoperative imaging techniques has considerably changed surgical strategies in neuro-oncology. We present an overview of clinical and radiological data of a mono-institutional retrospective cohort, focusing on the role of intraoperative multimodal imaging in surgical strategy. From 2015 to 2021, we identified 64 (81%) cranial (42 cerebellar, 8 supratentorial, and 14 of the brainstem) HBs and 15 (19%) spinal (4 cervical and 11 dorsal) HBs in 79 patients. Intraoperatively, indocyanine green videoangiography with FLOW800 was used in 62 cases (52 cranial and 10 spinal), intraoperative ultrasound and contrast-enhanced ultrasounds in 22 cases (18 cranial and 4 spinal HBs), and fluorescein in 10 cases (in 6 cranial and 2 spinal cases used as SF-VA). Gross total resection was achieved in 100% of the cases (53 mural nodule removal and 26 complete resections of the solid tumor). No side effects were reported following the combination of these tools. Multimodal intraoperative techniques provide valuable and reliable information to identify the tumor and its vasculature, guiding a more precise and safer resection and reducing the risk of recurrence.