Abstract
IntroductionSubarachnoid haemorrhage (SAH), secondary to spinal hemangioblastoma (HBL), is extremely rare, with only a few case reports to date. We report the experience of our reference centre for spinal tumours and Von Hippel–Lindau (VHL) disease in patients with spinal HBL presenting with SAH. We further performed a systematic review of the literature. MethodsWe report two cases. A systematic search was performed using the PubMed, Embase and Cochrane databases, with no limit for publication date. Inclusion criteria were: patients with HBL presenting with SAH, with or without VHL. The systematic review retrieved only 10 studies, including 16 patients. ResultsIn our centre, the first case concerned radicular HBL at D12 level, presenting with spinal and brain SAH. The patient underwent uneventful microsurgical en bloc resection. Postoperative course was normal. The second case concerned HBL with SAH at the cervico-medullary junction, with rapidly fatal course. The systematic review revealed female predominance, at a median age of 40 years, with HBL predominantly located at cervical level, common preoperative symptoms being headache and signs of meningeal irritation. ConclusionsIn conclusion, spinal HBL is an extremely rare cause of SAH. The systematic review found putative risk factors: female gender, age 40–50 years, cervical location, and median size 2cm. Diagnosis can be difficult when presentation mimics intracerebral SAH. We advocate early surgical removal. The risk of rapidly fatal course, in case of major haemorrhage, needs to be borne in mind.
Highlights
Subarachnoid haemorrhage (SAH), secondary to spinal hemangioblastoma (HBL), is extremely rare, with only a few case reports to date
HBL is sporadic (70 to 80% [7]), 20–30% of cases are related to a dominantly inherited genetic familial cancer syndrome known as von Hippel–Lindau (VHL) [6,8]
We further performed a systematic review of the literature for publications describing patients with HBL presenting with SAH, with or without surgical exploration, together with clinical and radiological outcomes
Summary
Subarachnoid haemorrhage (SAH), secondary to spinal hemangioblastoma (HBL), is extremely rare, with only a few case reports to date. We report the experience of our reference centre for spinal tumours and Von Hippel–Lindau (VHL) disease in patients with spinal HBL presenting with SAH. Hemangioblastomas (HBL) are highly vascularised lesions of the central nervous system (CNS), most frequently within the cerebellum and spinal cord [1,2,3]. They account for approximately 2–3% of primary spinal cord tumours [4]. They are considered benign (World Health Organisation grade I) [5]. Thirteen per cent of HBLs occur in the spinal canal [9]
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