Abstract
IntroductionIntracranial hemangioblastoma (HB) is a rare pathology. Limited data exist regarding its epidemiology.MethodsWith the SEER-18 registry database, information from all patients diagnosed with intracranial HB from 2004 to 2013 were extracted, including age, gender, race, marital status, presence of surgery, extent of surgery, receipt of radiation, tumor size, tumor location, and follow-up data. Age-adjusted incidence rates and overall survival (OS). Cox proportional hazards model was employed for both univariate and multivariate analyses.ResultsA total of 1307 cases were identified. The overall incidence of intracranial hemangioblastoma is 0.153 per 100,000 person-years [95% confidence interval (CI)=0.145–0.162]. Through univariate analysis, age < 40 [hazard ratio (HR)=0.277, p<0.001], no radiation [HR=0.56, p=0.047], and presence of surgery [HR=0.576, p=0.012] are significant positive prognostic factors. Caucasian race [HR=1.42, p=0.071] and female gender [HR=0.744, p=0.087] exhibit noticeable trends towards positive prognosis. Through multivariate analysis, younger age [HR=1.053, p < 0.01], race [HR=1.916, p<0.01], and presence of surgery [HR=0.463, p<0.01 were significant independent prognostic factors.ConclusionClinical factors such as younger age, Caucasian race, and presence of surgery are significant independent factors for overall survival in patients with HBs. Though analysis regarding extent of surgery did not produce a meaningful relationship, this may be related to surgical bias / expertise. Moreover, no validation for radiation therapy was identified, but this may be related to short follow up intervals and the variable growth patterns of HBs.
Highlights
Intracranial hemangioblastoma (HB) is a rare pathology
Though analysis regarding extent of surgery did not produce a meaningful relationship, this may be related to surgical bias / expertise
No validation for radiation therapy was identified, but this may be related to short follow up intervals and the variable growth patterns of HBs
Summary
Hemangioblastomas (HBs) are benign neoplasms (WHO grade I) that constitute roughly 2% of intracranial neoplasms and 2-10% of primary spinal cord neoplasms [1]. The pathology frequently arises below the tentorium, predominantly in the cerebellar hemispheres (up to 76%), near the brain stem, or along the spinal cord [2, 3]. These lesions can appear either sporadically (57–75%) or due to an association with von Hippel-Lindau (VHL) disease www.oncotarget.com (20–43%) [3]. Radiation therapy is an option for less accessible lesions, for multiple concurrent lesions, and for tumor control after subtotal resection. We scrutinized the database to evaluate the epidemiology of HBs
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