Pituitary stalk hemangioblastomas in von Hippel-Lindau disease.
OBJECTIVE
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 cm(3) (range 0.08-2.8 cm(3)). 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.
National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA. lonserr@ninds.nih.gov
Russell R
RR
Lonser
John A
JA
Butman
Ruwan
R
Kiringoda
Debbie
D
Song
Edward H
EH
Oldfield
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D018325:Hemangioblastoma / Q000175:diagnosis*; D006801:Humans; D007089:Image Enhancement; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010902:Pituitary Gland / Q000473:pathology; D010911:Pituitary Neoplasms / Q000175:diagnosis*; D015173:Supratentorial Neoplasms / Q000175:diagnosis*; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000175:diagnosis / Q000601:surgery*