Abstract
Background:Hemangioblastomas (HBLs) are benign neoplasms that contribute to 1-2.5% of intracranial tumors and 7-12% of posterior fossa lesions in adult patients. HBLs either evolve hereditarily in association with von Hippel–Lindau disease (vHL) or, more prevalently, as solitary sporadic tumors. Only few authors have reported on the clinical presentation and the neurological outcome of HBL.Methods:We retrospectively analyzed the clinical, radiological, surgical, and histopathologic records of 24 consecutive patients (11 men, 13 women; mean age 51.3 years) with HBL of the posterior cranial fossa, who had been treated at our center between 2001 and 2012. We reviewed the current literature, and discussed our findings in the context of previous publications on HBL. The study protocol was approved by the local ethics committee (14-101-0070).Results:Mean time to diagnosis was 14 weeks. The extent of resection (EOR) was total in 20 and near total in 4 patients. Four patients required revision within 24 h because of relevant postoperative bleeding. One patient died within 14 days. One patient required permanent shunting. At discharge, 75% of patients [n = 18, modified Rankin scale (mRS) 0-1] showed no or at least resolved symptoms. Mean follow-up was 21 months. Two recurrences were detected during follow-up.Conclusions:In comparison to other benign entities of the posterior fossa, time to diagnosis was significantly shorter for HBL. This finding indicates the rather aggressive biological behavior of these excessively vascularized tumors. In our series, however, the rate of complete resection was high, and morbidity and mortality rates were within the reported range.
Highlights
Hemangioblastomas (HBLs) are benign neoplasms that contribute to 1-2.5% of intracranial tumors and 7-12% of posterior fossa lesions in adult patients
Hemangioblastomas (HBLs) represent benign capillary‐rich neoplasms of the central nervous system (CNS) that are derived from a blood vessel lineage
HBLs account for 1-2.5% of intracranial tumors and 7-12% of posterior fossa lesions in adult patients.[21,32,33]
Summary
We retrospectively analyzed the clinical, radiological, surgical, and histopathologic records of 24 consecutive patients (11 men, 13 women; mean age 51.3 years) with HBL of the posterior cranial fossa, who had been treated at our center between 2001 and 2012. We reviewed the current literature, and discussed our findings in the context of previous publications on HBL. Between 2001 and 2012, 24 consecutive patients with HBL in the posterior cranial fossa underwent suboccipital craniotomy and consecutive microsurgical resection in our department. We retrospectively reviewed the patients’ clinical, neuroradiological, surgical, and histopathologic records. All patients were treated according to our standardized postoperative treatment regimen: (1). VHL was diagnosed on a solely clinical basis according to previous updated criteria.[8,23,30] Fundoscopy and abdominal echography or CT scans were carried out to screen for vHL The nature and duration of symptoms, the manifestations of vHL, and associated positive family history were evaluated by analyzing the patients’ charts. vHL was diagnosed on a solely clinical basis according to previous updated criteria.[8,23,30] Fundoscopy and abdominal echography or CT scans were carried out to screen for vHL
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