Pituicytoma is a rare hypophyseal tumour with very few case reports in literature so far. We report a 76 years old gentleman, with know hypertension, hypercholesterolemia and T2DM, who presented initially with features of hypogonadism and a bilateral inferior quadrantanopia. Initial Hormonal assessments revealed Testosterone of <0.05 nmol/l, FSH 2U/l, LH 2U/l and Prolactin 586 mU/l, confirming hypogonodotrophic hypogonadism. He was started on testosterone replacement using Striant SR 30 mg every 12 hours. Formal visual field assessment demonstrated bilateral inferior hemianopia and CT scans revealed a suprasellar lesion in keeping with a craniopharyngioma, above a normal looking pituitary gland. MR scans revealed a suprasellar enhancing lesion, extending posteriorly to the optic chiasm which appeared to be compressed. The pituitary appeared compressed but was otherwise normal.
A presumptive diagnosis of Craniopharyngioma was made. He underwent a transphenoidal hypophysectomy, which was uneventful. Post-operatively tests revealed pan hypopituitarism with LH<1.0 U/l, FSH<1.0 U/l; Testosterone <0.5 nmol/l, Prolactin 298 mU/l, TSH 0.12 mU/l,freeT4 of 9 pmol/l, and a short synacthen test with baseline 144, 30 minute level 293. He was started on full replacement therapy of thyroxine 50 mcg once a day and hydrocortisone 10/5/5 mg and Sustanon 250 mg. Histopathologically, the tumour was noted to be a pituicytoma which are rare benign primary tumours of neurohypophysis. Pituicytomas are spindle cell tumours with little nuclear pleomorphism. They are benign, low-grade, non-infiltrative neoplasm of unique glial elements referred to as pituicytes, arising in the neurohypophysis and within the sella turcica. Clinically, they may present as non functional pituitary tumours with mass effects, as in our patient. Little is known about the clinical course of these histologically benign tumours. Total resection may cure the tumour; however, subtotal resections are known to be prone to local recurrence and high vascularity and location make them surgically challenging.
01 - 05 Apr 2006
European Society of Endocrinology