Treatment of brainstem hemangioblastomas

Hemangioblastomas are benign vascular central nervous system (CNS) tumors. Hemangioblastoma are frequently associated with peritumoral cysts (cysts arising at tumor edge). Because resection is curative, it is the preferred therapy for symptomatic brainstem hemangioblastomas. Nevertheless, surgical removal can present a treatment challenge due to the delicate surrounding anatomic structures in this region of the CNS (Wang et al.2001; Wind et al.2011). Here, we describe the salient clinical and management features of brainstem hemangioblastomas. Hemangioblastomas are World Health Organization Grade I tumors of the central nervous system. Hemangioblastomas can present sporadically, and also comprise 5–10% of central nervous system (CNS) tumors in patients with von Hippel-Lindau (VHL) disease. Despite their sensitive location, brainstem hemangioblastomas can be removed safely; they generally should be resected when they become symptomatic or when the tumor has reached a size such that further growth will increase the risks associated with surgery, or in the presence of an enlarging cyst. Magnetic resonance imaging is usually sufficient for preoperative evaluation and presurgical embolization is unnecessary. The goal of surgery is complete resection of the lesion before the patient experiences a disabling neurological deficit.