Screening and surveillance recommendations for central nervous system hemangioblastomas in pediatric patients with Von Hippel-Lindau disease.

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.

Piezosurgery for safe and efficient petrous bone cutting in cerebellopontine angle and petroclival meningioma surgery.

Intradural petrous bone drilling has become a widespread practice, providing extended exposure in the removal of cerebellopontine angle (CPA) or petroclival tumors. Adjacent neurovascular structures are at risk, however, when drilling is performed in this deep and narrow area. Hence, this study evaluates the use of Piezosurgery (PS) as a non-rotating tool for selective bone cutting in CPA surgery. A Piezosurgery® device was used in 36 patients who underwent microsurgery for extra-axial CPA or petroclival tumors in our Neurosurgical Department between 2013 and 2019. The clinical and radiological data were retrospectively analyzed. The use of PS was evaluated with respect to the intraoperative applicability and limitations as well as efficacy and safety of the procedure. Piezosurgical petrous bone cutting was successfully performed in the removal of meningiomas or extra-axial metastases arising from the dura of the petroclival region (21 patients) or petrous bone (15 patients). PS proved to be very helpful in the deep and narrow CPA region, considerably reducing the surgeon's distress toward bone removal in close proximity to cranial nerves and vessels in comparison to common rotating drills. The use of PS was safe without injuries to neurovascular structures. Gross total resection was achieved in 67% of petroclival and 100% of petrous bone tumors. Piezosurgery proved to be an effective and safe method for selective petrous bone cutting in CPA surgery avoiding rotating power and associated risks. This technique can particularly be recommended for bone cutting in close vicinity to critical neurovascular structures.