Background: Supratentorial hemangioblastoma is an uncommon lesion with few data available. Resemblance to other tumours represents a struggle concerning pre-operative diagnosis and management.
Objectives: The aim of this study was to review the current literature, integrating two new cases with uncommon features.
Methods: A search of English language peer-reviewed articles in PubMed®, Cochrane Library®, Google Scholar® and summary of the current knowledge.
Results: A total of 162 cases, with no predominance between von Hippel-Lindau disease and wild-type sporadic lesions. The frontal lobe is the most common topography, followed by the pituitary stalk, with image resemblance to a glioma or a metastasis. From these, 20 cases revealed dural attachment, mimicking a meningioma. Symptoms are due to mass effect or epilepsy, with low haemorrhagic risk. Clinical outcome on supratentorial hemangioblastoma depends on resection extension, with no recognised complementary treatment. Post-operative follow-up is essential, even in wild-type, sporadic cases, concerning recent reports of tumour dissemination.
Conclusions: This review compiles the main characteristics of supratentorial hemangioblastoma, that despite its rarity, should be a concern on differential diagnosis, treatment planning and expected prognosis.
Keywords: Hemangioblastoma; leptomeningeal neoplasms; von Hippel–Lindau disease.