Suprasellar hemangioblastoma without von Hippel-Lindau disease: a case report and literature review.

Suprasellar hemangioblastoma (HBL) without von Hippel-Lindau (VHL) disease is extremely rare. A 51-year-old woman presented with headache and progressively deteriorating bilateral visual disturbance for 4 months. Magnetic resonance imaging (MRI) revealed a 2.5-cm solid mass in the suprasellar region with homogeneous contrast enhancement. Our preoperative presumptive diagnosis was meningioma. Resection of the tumor was achieved via a left pterional craniotomy. The tumor was reddish in appearance and relatively firm, and was extremely vascularized, which might provide extensive blood supply through small branches of the internal carotid artery. There was a clear border between the tumor and the pituitary stalk and optic nerves. Histopathologic examination showed that the tumor was well vascularized, consisting of a reticular mesh of numerous thin-walled capillaries and abundant stromal cells. Immunohistochemistry demonstrated the positive staining for CD34, vimentin (VIM), and neuron specific enolase (NSE) in the intratumoral capillaries, while negative staining of epithelial membrane antigen (EMA) and glial fibrillary acidic protein (GFAP) was observed. Based on these results, the patient was diagnosed as HBL. After the resection, the visual field defect in the left eye was markedly improved, and no tumor recurrence was noted in 1 year follow-up. When solid lesions are highly vascularized in the suprasellar region of patients, even though no VHL disease is present, the possibility of HBL should be taken into consideration. Moreover, craniotomy is a better treatment option for suprasellar HBL without VHL disease.