Case Report Recurrent Cerebellar Hemangioblastoma in Two Brothers Carrying Von Hippel Lindau Syndrome

Objectives: : To present two cases of cerebellar hemangioblastomas in two siblings, who received surgical treatment as first line treatment and currently underwent SRS in October 2024.

Methods: : This is an observatory descriptive case report study

Results: : Two family cases of cerebellar hemangioblastomas are presented in siblings from Lima, Peru. Both patients were carriers of Von Hippel-Lindau Syndrome. The initial approach for both patients was surgical treatment, and after having a recurrence at 5 and 4 years, respectively, stereotactic radiosurgery was chosen.

The first patient is a 34-year-old man, who presented nausea and vomiting in 2019 when he got diagnosed. In August 2019, he underwent surgery for a right cerebellar lesion. After that, he remained without any sign and symptoms after 5 years. However, a recurrence was found in the left contralateral hemisphere during a follow-up visit.

The second patient, a 32-year-old woman, had a right cerebellar lesion treated surgically in 2020. Before the procedure, the patient developed hydrocephalus, and a peritoneal ventricle shunt was placed. She remained without any symptom during the following 4 years, but in 2024 a recurrence was identified, consisting of a three-nodule lesion in the left cerebellar hemisphere.

On procedure day, the female patient’s extradural tumor volume was of 2.07 cm3. It received a dose of 20Gy at the margin with a 50% isodose surface and used 5 isocenters: one shot from the 14mm collimator and the other four using the 8mm one. In the case of the male patient the extradural intraspinal tumor had a target volume of 177.6 cm3. The dose at the margin was 20Gy with a 50% isodose surface and used 1 isocenter, from an 8mm collimator. In both cases the tolerance drop doses in the adjacent critical structures were non-significant.

Both patients remained with a Glasgow score of 15 and a Karnofsky score of 100 before and after the procedure. Postoperative management included a protocol of dexamethasone. It included an IBP before breakfast, 4mg of dexamethasone every 8 hours with gradual tapering for 5 days and paracetamol as needed for pain management.

During the first week of postoperative treatment patients remained asymptomatic. Currently they need to continue with their follow-up consults every 6 months for 2 years, followed by yearly visits for 5 additional years, every time with a new MRI.

Conclusion(s):: This report highlights the infrequent occurrence of the Syndrome of Von Hippel Lindau in two siblings. Furthermore, both patients having a similar progression of treatment and recurrence of the tumors.