Metachronous Pituitary Stalk And Cerebellar Hemangioblastomas In A Patient Without Von Hippel-Lindau Disease: Case Report And Review Of Literature

Background: Hemangioblastomas (HBs) are benign neoplasms of the central nervous system (CNS) that account for 10–15% of posterior fossa tumors in adults. HBs most commonly arise in the cerebellum but also occur in the spinal cord and medulla. They occur sporadically as solitary lesions or in association with von Hippel-Lindau (VHL) disease and are usually multiple. HBs can arise in the supratentorial space (>100 reported cases) but only 16 cases of pituitary stalk HBs have been reported—87.5% of which occurred in the setting of VHL disease. Although multiple, metachronous and disseminated HBs can occur in the setting of VHL disease, there are no reported cases of focal, metachronous HBs in patients without this disease. Case Description: We report the case of a 61-year old man with no personal or family history of VHL disease who presented with a pituitary stalk HB 9 years after complete resection of a cerebellar HB. He underwent complete resection complicated by diabetes insipidis and visual field deterioration.Conclusion: The occurrence of HB at different times and in disparate locations within the CNS is not synonymous with the diagnosis of VHL disease. While diffuse dissemination has been reported following surgery, this is the first reported case of a focal metachronous HBs arising in a patient without VHL disease. Although its unusual location within the pituitary stalk complicated the diagnosis preoperatively, a second HB should be considered in the differential diagnosis and close follow-up may be warranted to identify such lesions prior to irreversible neurological deficits.