Pituitary stalk hemangioblastomas in von Hippel–Lindau disease

Abstract

Object Pituitary stalk hemangioblastomas are rare, and information on them is limited to a small number of case reports. To gain insight into the incidence, clinical effects, and management of pituitary stalk hemangioblastomas, the authors analyzed a series of patients with von Hippel–Lindau (VHL) disease. Methods Patients with VHL disease who were enrolled in a prospective National Institutes of Health natural history study were included. Clinical, imaging, and laboratory findings were analyzed. Results Two hundred fifty patients were included in the study (120 male and 130 female patients). In 8 patients (3%), 8 pituitary stalk hemangioblastomas were identified on MR imaging. This anatomical location was the most common supratentorial site for these lesions; 29% of all supratentorial hemangioblastomas were found there. The mean (± standard deviation) pituitary stalk hemangioblastoma volume was 0.5 ± 0.9 cm3 (range 0.08–2.8 cm3). Results of endocrine laboratory profiles were normal in all patients. All patients remained asymptomatic and none required treatment during the follow-up period (mean duration 41.4 ± 14.4 months). Conclusions The pituitary stalk is the most common site for the development of supratentorial hemangioblastomas in patients with VHL disease. Pituitary stalk hemangioblastomas often remain asymptomatic and do not require treatment. These findings indicate that pituitary stalk hemangioblastomas in patients with VHL disease may be managed with observation and that surgery for them can be reserved until associated signs or symptoms occur.

Pituitary Stalk Hemangioblastoma in a von Hippel-Lindau Patient : Clinical Course Follow-Up Over a 20-Year Period

“…Cerebellar HBs are the most common manifestation of VHL disease and are often multiple in nature3,4). Although cerebellar HBs are histopathologically benign, they show a degree of malignant clinical behavior in long-term follow up.…”

“…HBs originate most frequently in specific regions of the CNS (retina, cerebellum, brainstem, and spinal cord) and seldom occur in the supratentorial compartment of the CNS3). In a recent series, 11% of the HBs in VHL disease were developed in supratentorium, which is more than previously thought5).…”

“…Hemangioblastomas (HBs) are benign vascular tumors of the central nervous system (CNS) that occur either sporadically (67-80% of cases) or as part of von Hippel-Lindau disease (VHL, 20-33% of cases)3,5,6). HBs originate most frequently in specific regions of the CNS (retina, cerebellum, brainstem, and spinal cord) and seldom occur in the supratentorial compartment of the CNS3).…”

Rathke's cleft cysts in twins with Type 2C von Hippel-Lindau disease

“…6 Suprasellar and intrasellar hemangioblastomas have been documented. [9][10][11] There is one case report of pituitary adenoma and paraganglioma in a patient with VHLD. 17,18 Rathke's cleft cysts are benign, cystic remnants of the embryological stomodeum.…”

15 YEARS OF PARAGANGLIOMA: The association of pituitary adenomas and phaeochromocytomas or paragangliomas

“…Lesions within and around the sella can mimic PAs and might be coincidental, for example, Rathke's cleft cyst in VHL (Huff et al 2014), related to a particular syndrome, such as haemangioblastomas in VHL (Goto et al 2001, Lonser et al 2009, Kanno et al 2013, or the other pathology as in the case of an intrasellar PGL (Boari et al 2006).…”