Spontaneous Hemorrhage from Central Nervous System Hemangioblastomas

Objective

Hemagioblastomas are highly vascular tumors that rarely present with hemorrhage. To date, the only factor reported to possibly influence the propensity of a tumor to bleed has been its size (>1.5 cm). Here, we present our series of hemorrhagic hemangioblastomas, which includes 2 very small tumors (<1.5 cm). We propose that other factors apart from size may predispose tumors to hemorrhage.

Methods

We conducted a retrospective analysis of 55 cases of central nervous system hemangioblastomas operated on at Harborview Medical Hospital and the University of Washington between 2004 and 2014. Demographical and clinical data were collected and analyzed to determine factors that may predispose these tumors to hemorrhage.

Results

Of 55 cases, only 3 patients presented with hemorrhage (5.5%). None of the patients were von Hippel-Lindau positive. Two of the hemorrhagic tumors were less than 1.5 cm, countering previous studies in which authors proposed that hemangioblastomas that are less than 1.5 cm have essentially no risk of hemorrhage. One tumor also rebled after preoperative embolization.

Conclusions

Our series suggests that small hemangioblastomas may represent a hemorrhagic risk. We speculate that other factors, such as genetic predisposition caused by single-nucleotide polymorphisms, may play a role in hemangiobalstomas that present with rupture.

Introduction

Central nervous system (CNS) hemangioblastomas are highly vascular tumors typically found in the posterior fossa or medullary substance of the spinal cord and represent approximately 2%–3% of all CNS tumors (13). Hemangioblastomas may be associated with von Hippel-Lindau (VHL) syndrome, where they occur with a prevalence of 20%–30% (21). Presenting symptoms of cerebellar hemangioblastomas typically relate to hydrocephalus (headache, nausea, vomiting, diplopia, etc) whereas spinal hemangioblastomas typically present with focal neurologic deficit related to mass effect. On very rare occasions, hemangioblastomas of the brain or spine may present with spontaneous subarachnoid, intra-ventricular, or intra-parenchymal hemorrhage. Fifty-three such cases reports exist in the literature 2, 3, 4, 5, 6, 7, 8, 9, 11, 12, 13, 14, 15, 16, 17, 19, 20, 22, 24, 25, 26, 28, 29, with only one of these studies attempting to systematically evaluate risk factors that may predispose certain hemangioblastomas to spontaneous hemorrhage (13). Glasker et al. 12, 13 postulated that size alone was the most important risk factor for spontaneous hemorrhage, concluding that tumors <1.5 cm pose essentially no risk of hemorrhage. Here, we challenge this hypothesis by presenting our series of hemorrhagic hemangioblastomas, 2 of which were smaller than 1.5 cm in maximal diameter. The potential pathophysiology of hemorrhage in these tumors is also discussed.