Introduction: Supratentorial hemangiblastomas are a a very rare tumor. There is little information about it incidence, natural history, prognosis and best treatment. We present a case of a sporadic supratentorial hemangioblastoma with a distinct location, frontal lobe, and a review of the pertinent literature about this theme.
Methods: A patiente of 45 years old with new onset of headache was submited to a surgery because a mass in the frontal lobe. After a histopathologic confirmation of hemangioblastoma we made a review of the related literature and discuss our case.
Results: The patient harboed a mass of 22 cc of volume in the right frontal lobe closed with the midline. The pathology confirmed that was a hemangioblastoma and the patient was submitted to a genetic analisis showing that he doesnt have Von Hippel Lindau Disease. In this scenario we confirmed a rare tumor in a very rare location possibly one of the first in the literature. Our review showed that supratentorial hemangioblastoma are a very rare disease with conflicted data regarding their incidence, natural history, prognosis and treatment.When dealing with sporadic supratentorial hemangioblastomas exist only few cases reported and they are located specially in the selar/suprasselar region and temporal lobe. Probably this is one of the first cases of this tumor in the frontal lobe.
Conclusions: We conclude that supratentorial hemangioblastomas are very rare tumors with poor description in the literature about their natural history, incidence and clinical aspects but closed related to Von Hippel Lindau disease. Sporadic supratentorial hemangioblastomas are very rare as this case we present and specially in the frontal lobe this is one of the first cases.
Patient Care: After review of the case we had a better understanding of his disease and could make a better prognosis and orientation for him.
Learning Objectives: Discuss the clinical aspects of supratentorial hemangioblastomas and present and unusual case with a unusual presentation.