Supratentorial hemangioblastomas: differences in phenotypes are correlated with the anterior vs. the posterior cerebral circulations

Abstract

Abstract Background Supratentorial hemangioblastomas (ST HB) are rare, vascular lesions. The presence of peri-tumoral cysts and edema has meaningful clinical, diagnostic and therapeutic implications. Nevertheless, the pathogenesis of both cyst and edema formation is not fully understood. Aim to investigate whether the radiologic phenotype of ST HB is affected by the different cerebral arterial circulations. Material and methods We have reviewed the published English literature over the last 5 decades (January 1973 to January 2023). 73 cases of parenchymal ST HB were eligible for analysis. The cohort was divided into two groups based on the vascular territory distribution of the lesions: anterior circulation (group 1, n = 52) vs. posterior circulation (group 2, n = 21). The two subgroups were compared for demographic, clinical, radiological and molecular variables. Results On univariate analysis, male gender and cystic changes were associated with HB of the posterior circulation (OR = 3.61, p = 0.024 and OR = 4.85, p = 0.01; respectively). On the other hand, significant peritumoral edema and parasagittal/paramedian location were associated with the anterior circulation (OR = 0.18, p = 0.05 and OR = 0.30, p = 0.036; respectively). On multivariate analysis, cystic changes have remained significantly associated with the posterior circulation, while the association of significant peritumoral edema with the anterior circulation has nearly reached statistical significance (OR = 5.47, p = 0.008 and OR = 0.25, p = 0.054). The incidence of von-Hippel Lindau (vHL) disease was nearly two-fold higher in group 1. In group 2, all (100%, n = 7) female patients had cystic tumors, compared to only 30.7% (n = 8) of female patients in the anterior circulation group (OR = 32.647, 95% CI 1.665 to 640.016; p = 0.0217). Conclusion based on historical cases of ST HB, we were able to show that different tumor phenotypes are associated with the different cerebral circulations. In addition, gender was also associated with differences in tumor’s distribution as well as radiologic phenotype. Together, this novel data may influence our understanding on other unique vascular diseases of the central nervous system.