Current Diagnostic and Therapeutic Strategies in Treatment of CNS Hemangioblastomas in Patients with VHL - Abstract
Hemangioblastomas are a rare form of benign vascular tumors of the CNS. They can occur sporadically or as component of the von Hippel-Lindau (VHL) disease - an autosomal dominant tumor syndrome. The tumors are typically located in the posterior fossa and spinal cord. Patients with associated VHL disease are usually affected at an early age and develop multiple lesions. Therefore they need a special routine for diagnosis, treatment and follow-up strategies.
In modern neurosurgery, hemangioblastomas are well resectable tumors. Symptomatic lesions should be removed. Resection should furthermore be considered for asymptomatic progressive tumors for the following reason: If a tumor has already caused neurological deficits, the chance to reverse these by surgical resection is reduced and surgical resection is usually possible with low morbidity. Preoperative embolization of large solid hemangioblastomas prevents perioperative hemorrhage but is not necessary in every case. Surgical strategies depend on many factors such as timing of diagnosis, location and progress of tumor growth and have to be discussed individually. Radiation or chemotherapy should be reserved for inoperable tumors.
The University of Freiburg serves as a reference center for VHL disease. Here we present the current diagnostic and treatment strategies of hemangioblastomas - based on our own experiences and by review of renowned literature on this topic.