Pituitary Stalk Hemangioblastoma in a von Hippel-Lindau Patient : Clinical Course Follow-Up Over a 20-Year Period
A 15-year-old boy was admitted to our hospital in 1990 with an one year history of intermittent headache and vomiting. A solitary hypervascular mass was demonstrated at the right cerebellum and was removed completely. Histologic finding was consistent with HB. There was no recurrence during a few years of follow-up. However, 7 years following the initial surgery, brain magnetic resonance image (MRI) showed a cystic mass with a mural nodule in the right cerebellar hemisphere and several small masses at the right cerebellopontine angle and left cerebellar vermis representing recurrence. Resection of the lesion was achieved, using suboccipital craniectectomy, and gamma-knife therapy was performed. Seventeen years following the initial surgery (2007), he developed progressive headache, dizziness and diplopia. At that moment, brain MRI revealed a homogeneous enhancing suprasellar mass () and enlarged edematous optic chiasm (). Additionally, he was clinically diagnosed with VHL disease with numerous enhancing nodules and syringomyelia along the cervical and thoracic spine, multiple cysts in the both kidneys and the pancreas, and renal cell carcinoma in the lower pole of the right kidney. His endocrinological profile was normal. He subsequently received gamma-knife radiosurgery for pituitary and other residual lesions in the brain. Twenty-two years following the initial surgery (2012), he complained recurred dizziness and progressive diplopia. Brain MRI revealed an increased size of the pituitary mass (from 4.3×5 mm to 7×11.5 mm) and cerebellar masses, despite treatment. After the cerebellar lesion has been removed surgically, he was discharged and scheduled for further follow-up imaging and gamma-knife therapy because the patient's symptoms were subsided and the surgeon opted for observation.