Meningeal Supratentorial Hemangioblastoma in a Patient with Von Hippel-Lindau Disease Mimicking Angioblastic Menigioma
A 51-year-old female patient presented to our hospital with severe dizziness and right facial paresthesia. Five years previously, magnetic resonance imaging (MRI) had shown 2 small masses on the right cerebellum and 1 large mass on the left cerebellum. At that time, lateral vertebral artery digital subtraction angiography (DSA) had shown a highly vascular tumor nodule supplied by the posterior inferior cerebellar artery. Lateral internal carotid artery (ICA) and external carotid artery (ECA) DSA had shown no vascular abnormalities. T1-weighted gadolinium enhancement MRI had shown no abnormal findings in the supratentorial region (). The patient had undergone craniotomy, and the left cerebellar tumor was removed. The pathological diagnosis was HB. Abdominal computed tomography, an evaluation of the patient's family history, and genetic screening had been employed to evaluate the presence of VHL disease. Computed tomography had revealed cysts in the pancreas, liver, and both kidneys. Three of the patient's 6 siblings and 1 of her 2 children were diagnosed with VHL disease. Genetic analysis had shown the presence of a VHL gene mutation; thus, the patient's condition had been diagnosed as HB associated with VHL disease.\nFollow-up MRI performed 1 and 12 months after the first operation had shown no evidence of recurrence or abnormal findings in the supratentorial region. However, MRI performed 5 years after the first operation showed a 4.0×3.6×4.0-cm cystic mass with strong enhancement, dural base thickening, and peritumoral edema on the left frontal surface. However, no tumor recurrence was observed in the cerebellum. Left ICA DSA showed pial blood supply to the rim of the tumor. Left ECA DSA showed tumor staining from the middle meningeal artery ().\nThe tumor was completely removed after preoperative embolization. It was a vascular tumor with extensive dural attachment. Histopathological examination with hematoxylin and eosin staining showed lipid-containing vacuoles and abundant vascular cells (). The tumor cells were negative for epithelial membrane antigen (EMA), glial fibrillary acidic protein (GFAP), CD34, and S100, but positive for neuron-specific enolase (NSE) and vimentin (). The patient was definitively diagnosed with HB with meningeal involvement. The patient's dizziness resolved postoperatively. There was no recurrence of the supratentorial tumor or change in the size of the multiple small right cerebellar tumors during the 12-month follow-up period.