Suprasellar Hemangioblastoma Unrelated to von Hippel-Lindau Disease Successfully Treated through Extended Transsphenoidal Approach: Diagnostic Value of Von Hippel-Lindau Disease Gene-Derived Protein

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Hemangioblastoma tends to occur in the infratentorial regions and rarely in the supratentorial regions. This tumor is strongly associated with von Hippel-Lindau disease (VHLD), especially in the supratentorial regions, with only 12 cases of suprasellar hemangioblastoma unrelated to VHLD. However, all these cases were diagnosed based on clinical screening and confirmation of family history. We report a case of suprasellar hemangioblastoma that was successfully removed through an extended transsphenoidal approach and diagnosed as a sporadic case unrelated to VHLD by immunohistochemical examination.

A 67-year-old woman had mild diabetes insipidus and a visual field defect. Head magnetic resonance imaging revealed a suprasellar tumor compressing the optic chiasm upward. The tumor was totally removed without complication through an extended transsphenoidal approach. Postoperative histologic examination disclosed large vacuolated stromal cells and rich capillary networks, and the diagnosis was established as hemangioblastoma. Clinical screening and confirmation of family history revealed no specific results, and additional immunohistochemical staining showed diffuse cytoplasmic expression of anti-VHLD gene-derived protein (pVHL).

We emphasize that pVHL immunohistochemical evaluation would be wise to adopt, especially for patients with a high risk of VHLD related to younger age and supratentorial lesions.

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