Endocrine Abstracts

Introduction: Hemangioblastomas (HBL) in the pituitary stalk are extremely rare. Most pituitary stalk HBL reported in the literature were associated with von Hippel–Lindau (VHL) disease.

Case report: We report the case of a 34-year-old female patient with VHL disease diagnosed at the age of 18 with multiple complications (bilateral retinal and cerebellum HBL, renal cell carcinoma). Brain magnetic resonance imaging (MRI) demonstrated an hypointense suprasellar mass measuring 9×8×9 mm with marked homogenous contrast enhancement after gadolinium administration, originating from the pituitary stalk, suggesting a pituitary stalk HBL. The initial hormonal workup was normal. A visual field defect evaluation was not feasible due to severely low bilateral visual acuity. During the 4-year follow-up, a slight increase in the mass volume and prolactin level was observed; depending on the clinical, radiological and laboratory evolution, a future surgical approach cannot be disregarded.

Review of the literature: The previously reported cases of HBL in the pituitary stalk were analyzed. Seventeen cases have been reported, the majority (fourteen) being associated with VHL disease and the remaining considered to be sporadic. Ten patients had no visual symptoms or hormonal dysfunction, and therefore were kept on observation. A total of six patients were operated. Of the six surgical cases, four patients developed panhypopituitarism, and two patients had visual field disturbances preoperatively with partial recovery after surgery. None of the patients were treated with radiotherapy.

Conclusion: To our knowledge, this is the 18th case of HBL in the pituitary stalk. According to our review, pituitary stalk HBL often remain asymptomatic and do not require treatment. Surgery can be reserved until associated signs or symptoms occur.