NCMP-07. HEADACHE PRESENTATION AND MANAGEMENT IN VON HIPPEL-LINDAU (VHL)-ASSOCIATED HEMANGIOBLASTOMAS

Abstract

Abstract BACKGROUND Hemangioblastomas are the most common brain tumors among patients with Von Hippel-Lindau (VHL) disease. Common characteristics of these benign tumors, such as tumor growth, peritumoral edema, and cystic expansion, may lead to neurologic morbidity and/or mortality. Thus, early diagnosis based on symptom presentation and appropriate treatment is paramount to VHL-associated hemangioblastoma management. METHODS We conducted a systematic review of PubMed, Scopus, Cochrane, Embase, and Web of Science databases using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We identified studies reporting pre-diagnostic symptoms and post-diagnostic treatments among patients with VHL-associated hemangioblastomas. RESULTS Data from 150 patients were analyzed. The average age at presentation was 35.3 years (range: 18-75) and 52.7% of patients were female. Headache was the most common presenting symptom (73.3%). Other presenting symptoms included balance or gait disturbances (42%), ataxia (41.3%), nausea (24.3%), emesis (24.3%), dysmetria (15.5%), swallowing difficulties (11.3%), and singultus (10.7%). Anatomically, 98.7% of tumors were in the posterior fossa, specifically 69% in the cerebellum and 30% in the brainstem. Approximately 62.8% of patients had tumor associated cysts. A majority of the patients (98.7%) underwent gross-total resection of the tumor and cyst drainage as needed. Other common treatments included radiotherapy, gamma-knife surgery, and ventriculoperitoneal shunt placement. CONCLUSION Patients with VHL-associated hemangioblastomas commonly presented with headaches, balance or gait difficulties, and ataxia. Tumors were generally found in the posterior fossa and were associated with cysts. Gross-total resection was the main treatment offered to patients with these tumors.