Sporadic supratentorial hemangioblastoma with meningeal affection: A case report and literature review

Abstract

Background: Hemangioblastomas are vascular tumors, of benign behavior, that originate in the central nervous system. Supratentorial hemangioblastomas are extremely rare and are generally associated with Von Hippel-Lindau disease (VHL). The involvement of structures by contiguity, such as blood vessels or meninges, is something exceptional. There are few references in the literature of supratentorial hemangioblastomas with meningeal involvement and most of them are described in reports or small case series. Methods: We performed a systematic review of the literature to observe the characteristics of patients with supratentorial hemangioblastomas with meningeal involvement. In addition, we present the case of a 43-year-old male patient with a sporadic supratentorial hemangioblastoma with both, meningeal and vascular involvement that recurred years after treatment. Results: The patients presented supratentorial tumors with meningeal involvement, we had a 1.2:1 ratio male-female distribution. The mean age was 50 years. Most tumors were located in the cerebral hemispheres, the lobe affected most frequently was the frontal lobe. About 67% of the cases were sporadic and only 21% were related to VHL disease. There were three cases of recurrence after surgery. Conclusion: Supratentorial hemangioblastomas are extremely rare lesions. It is rare for supratentorial hemangioblastomas to invade adjacent structures such as blood vessels or meninges, however, when this happens, it is recommended a preoperative vascular imaging study, especially in parasagittal hemangioblastomas where superior longitudinal sinus may be involved. In these cases, en bloc surgical resection is difficult and the probability of recurrence is higher. Although clinical trials and studies with a greater casuistry are necessary to establish guidelines for the management of supratentorial hemangioblastomas, nowadays, contribution of new cases is useful for understanding this pathology.