Cerebellar Hemangioblastoma in the Setting of Von Hippel-Lindau Disease Presenting as Cardiac Arrest
Document Type
Conference Proceeding - Restricted Access
Publication Date
5-8-2026
Abstract
Von Hippel-Lindau (VHL) disease is an autosomal dominant tumor predisposition syndrome characterized by benign and malignant neoplasms, including hemangioblastomas of the retina, brainstem, cerebellum, and spinal cord, as well as renal cell carcinoma and pancreatic lesions. Central nervous system (CNS) hemangioblastomas in VHL are typically detected through scheduled surveillance imaging or investigation of chronic neurologic symptoms rather than acute decompensation. This report describes a rare presentation of presumed cerebellar and cervical spinal cord hemangioblastomas manifesting as out-of-hospital cardiac arrest in a young adult with known VHL but no prior CNS involvement.
A 19-year-old woman with a history of VHL, previously noted to have only a pancreatic cyst and retinal "spots" on surveillance imaging, presented via EMS after an unwitnessed out-of-hospital cardiac arrest preceded by dyspnea, subjective paralysis, and distal numbness during a phone call. Return of spontaneous circulation was achieved after approximately one and a half rounds of chest compressions without defibrillation or medications, and she arrived at the emergency department hemodynamically stable but with depressed mental status (GCS 10) and no focal complaints owing to altered responsiveness. Initial evaluation, including point-of-care ultrasound, broad laboratory testing, and trauma and toxicologic workup, did not identify a cardiopulmonary or metabolic cause; shortly thereafter, she deteriorated with hypotension, bradycardia, and hypoxia during CT imaging, prompting emergent intubation and further neurologic evaluation.
This case highlights that VHL-associated CNS hemangioblastomas, although usually identified on surveillance imaging, can rarely present with sudden, life-threatening neurologic compromise mimicking primary cardiopulmonary causes such as cardiac arrest. Emergency medicine clinicians should maintain a general knowledge of genetic conditions and consider them in the differential diagnosis of atypical presentations.
Recommended Citation
Nisper E, Barnes M, Singh M. Cerebellar hemangioblastoma in the setting of Von Hippel-Lindau disease presenting as cardiac arrest. Presented at: Research Day Corewell Health West; 2026 May 8; Grand Rapids, MI.
Comments
2026 Research Day Corewell Health West, Grand Rapids, MI, May 8, 2026. Abstract 1920