Introduction: Hypophysitis is an inflammatory disease of the pituitary gland that may mimic pituitary tumors clinically and radiologically. Primary hypophysitis has traditionnaly been classified as lymphocytic (LH), granulomatous (GH), and xanthomatous (XH).
Case description: We report on a case of a xanthomatous hypophysitis initially diagnosed as pituitary adenoma. A 23-year-old men suffered from typical cluster type headache. Two years after the first symptoms, we confirmed diabetes insipidus in the background of polyuriapolydipsia. All the anterior pituitary hormone levels were normal. Head MRI scan depicted a 14×10×17 mm inhomogenous mass int he sella. Transphenoidal surgery was performed; the removed tissue showed by accumulation of foamy cells and xanthomatous epithelioid cells. The patient had an uneventful postoperative recovery. After surgery, we tried to stop the hydrocortisone therapy but severe cluster type headache returned. The endrocrine work up revealed hypadrenia (morning cortisol: 96 nm/l, ACT: 3.38 pm/l), hypothyroidism (ft4: 10.5 pm/l), hypogonadism (testosterone: 3.44 nm/l) with normal FSH and LH (FSH: 3.3 mIU/l, LH: 2.8 mIU/l). We restarted hormonal therapy: hydrocortisone, L-thyroxine and testosterone were stepwise reintroduced. During the follow-up period we could stop the hydrocortisone and L-thyroxine supplementations, whereas the patient has permanently required desmopressin and testosterone substitution. Because of his hypogonadism after pituitary surgery, the patient was sent for sperm storage. Control sella MRI scans revealed no progression of the intitially seen pitiutary tumor.
Conclusion: We describe an unusual case of xanthomatous hypophysitis causing cluster type headache and permanenty requiring ddAVP (desmopressine) and testostreone supplementatation however, without need for maintenance medication with hydrocortisone and L-thyroxine.
27 Apr - 01 May 2013
European Society of Endocrinology