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Development of a small solid cerebellar haemangioblastoma into a large pseudocyst with a mural nodule in a patient without VHL; the importance of regular follow-up


Haemangioblastomas (HBLs) are rare central nervous system tumours accounting for only 1.2–2.5% of all intracranial tumours. While most HBLs occur sporadically, 36–40% of cases are associated with von Hippel-Lindau (VHL) syndrome. Owing to its benign nature, sporadic cases are usually detected only when symptoms occur due to mass effect. Thus, the natural history of HBLs has only been studied in association with VHL syndrome. We present a case of sporadic HBL with a rapid evolution of its small nodule into an enlarging mural nodule with a large pseudocyst that resulted in increased intracranial pressure. Craniotomy for complete tumour removal was performed and the patient fully recovered. This case implies a regular follow-up of HBL might be mandatory even in patients without VHL.

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