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Background: Hemangioblastomas are highly vascularized tumors that may be associated with extensive architecture of the surrounding pathological vessels. The distinction between feeding arteries and draining veins is usually not obvious during microsurgical en-bloc tumor resection. The aim of this investigation is to provide recommendations in which hemangioblastomas intraoperative indocyanine green (ICG) videoangiography might be beneficial for safe en-bloc tumor resection., Methods: This is a single-center retrospective review of resected spinal hemangioblastomas over a 59-month period to identify operations in which ICG videoangiography was used. We analyzed whether intraoperative ICG videoangiography is useful for identifying possible feeding arteries and draining veins. The identified benefits and shortcomings of this technique were summarized., Results: In total, 39 patients had surgery for removal of spinal hemangioblastomas. Intraoperative ICG videoangiography was performed in 26 surgeries for resection of spinal hemangioblastomas (66.7 %). In 25 of 27 removed hemangioblastomas (92.6 %), intraoperative ICG videoangiography yielded useful insights about the vascularization of the tumor and as thus regarded as helpful. In two cases, the pathological vessels could not be clearly assigned to feeding arteries or draining vessels. Complete tumor removal was achieved in all patients., Conclusion: ICG videoangiography offers real-time intraoperative visualization of the tumor vasculature and can therefore improve surgical decision-making. Ideally, direct microscopic visualization of the structures to be assessed should be aimed for in ICG videoangiography. The information gained from ICG videoangiography may be limited in the case of tumors or vessels that lie deeper or are covered by the myelon or other structures., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Purpose: Von Hippel-Lindau (VHL) disease is an autosomal-dominantly inherited tumor predisposition syndrome. One of the most common tumors are central nervous system (CNS) hemangioblastomas. Recommendations on the initiation and continuation of the screening and surveillance program for CNS tumors in pediatric VHL patients are based on small case series and thus low evidence level. To derive more robust screening recommendations, we report on the largest monocentric pediatric cohort of VHL patients., Methods: We performed a retrospective analysis on a pediatric cohort of 99 VHL patients consulted at our VHL center from 1992 to 2023. Clinical, surgical, genetic, and imaging data were collected and statistically analyzed., Results: 42 patients (50% male) developed CNS hemangioblastomas, of whom 18 patients (56% male) underwent hemangioblastoma surgery (mean age at first surgery: 14.9 ± 1.9 years; range 10.2-17). The first asymptomatic patient was operated on at the age of 13.2 years due to tumor progress. Truncating VHL mutation carriers had a significantly higher manifestation rate (HR = 3.7, 95% CI: 1.9-7.4, p < 0.0001) and surgery rate (HR = 3.3, 95% CI: 1.2-8.9, p = 0.02) compared with missense mutation carriers., Conclusion: We recommend starting MRI imaging at the age of 12 years with examination intervals every (1-) 2 years depending on CNS involvement. Special attention should be paid to patients with truncating variants. Affected families should be educated regularly on potential tumor-associated symptoms to enable timely MRI imaging and eventually intervention, as CNS hemangioblastoma may develop before screening begins., German Clinical Trials Register Registration Number: DRKS00029553, date of registration 08/16/2022, retrospectively registered., (© 2024. The Author(s).)

Introduction: Hemangioblastomas are rare, histologically benign, highly vascularized tumors of the brain, the spinal cord, and the retina, occurring sporadically or associated with the autosomal dominant inherited von Hippel-Lindau (VHL) disease. Children or adults with VHL disease have one of > 300 known germline mutations of the VHL gene located on chromosome 3. They are prone to develop hemangioblastomas, extremely rarely starting at age 6, rarely at age 12-18, and, typically and almost all, as adults. There is a plethora of VHL-associated tumors and cysts, mainly in the kidney, pancreas, adrenals, reproductive organs, and central nervous system. Due to a lack of causal treatment, alleviation of symptoms and prevention of permanent neurological deficits as well as malignant transformation are the main task. Paucity of data and the nonlinear course of tumor progression make management of pediatric VHL patients with hemangioblastomas challenging., Methods: The Freiburg surveillance protocol was developed by combining data from the literature and our experience of examinations of > 300 VHL patients per year at our university VHL center., Results: Key recommendations are to start screening of patients at risk by funduscopy with dilated pupils for retinal tumors with admission to school and with MRI of the brain and spinal cord at age 14, then continue biannually until age 18, with emergency MRI in case of neurological symptoms. Indication for surgery remains personalized and should be approved by an experienced VHL board, but we regard neurological symptoms, rapid tumor growth, or critically large tumor/cyst sizes as the key indications to remove hemangioblastomas. Since repeated surgery on hemangioblastomas in VHL patients is not rare, modern neurosurgical techniques should encompass microsurgery, neuronavigation, intraoperative neuromonitoring, fluorescein dye-based intraoperative angiography, intraoperative ultrasound, and minimally invasive approaches, preceded in selected cases by endovascular embolization. Highly specialized neurosurgeons are able to achieve a very low risk of permanent morbidity for the removal of hemangioblastomas from the cerebellum and spinal cord. Small retinal tumors of the peripheral retina can be treated by laser coagulation, larger tumors by cryocoagulation or brachytherapy., Conclusion: We consider management at experienced VHL centers mandatory and careful surveillance and monitoring of asymptomatic lesions are required to prevent unnecessary operations and minimize morbidity.