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  • Title: Supratentorial hemangioblastomas in von Hippel-Lindau wild-type patients - case series and literature review.
    Author: Rocha L, Noronha C, Taipa R, Reis J, Gomes M, Carvalho E.
    Journal: Int J Neurosci; 2018 Mar; 128(3):295-303. PubMed ID: 28967275.
    Abstract:
    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.
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