Endocrine Abstracts

Purpose: The authors review the clinical outcomes of patients with hypophysitis.

Methods: Medical records of hypophysitis patients who were followed between 2007 and 2018 at Cerrahpasa Medical Faculty were evaluated retrospectively. Clinical, endocrinological, pathological and radiological findings and therapies were assessed.

Results: Twenty patients (F/M:12/8) were identified with a mean age at diagnosis of 39.14±17.78 years. The mean follow-up was 34.23±14.12 months. Eleven out of 20 patients (55%) were diagnosed histopathologically, 9 patients (45%) were diagnosed clinically with typical MRI findings. Five of 20 patients (25%) were secondary hypophysitis: 3 histiocytosis-X, 1 neurosarcoidosis and 1 Erdheim Chester disease. From 7 histologically diagnosed patients with primary hypophysitis, 5(25%) had lymphocytic, 1(5%) had lymphocytic-granulomatous and 1 (5%) had xanthomatous hypophysitis. None of the hypophysitis cases were diagnosed after pregnancy. The most commonly seen symptoms are headache (65%), polyuria/polydipsia (45%) and fatigue (%30). Pre-treatment endocrinological evaluation revealed that 9 (57%) patients had panhypophysitis, 9 (45%) had diabetes insipidus, 6 (30%) had hyperprolactinemia, 7 (35%) had isolated endocrine deficiencies with partial gland function and 2 (10%) had normal laboratory values. Radiologic findings of patients at the time of diagnosis revealed various results including large sellar mass (65%), thickened infundibulum (50%), uniform contrast enhancement (40%), loss of hypophysis bright spot on T1 imaging (20%) and partial empty cella (15%). Six out of 15 primary hypophysitis patients were treated conservatively and six of them had been operated. The remaining 3 out of 15 and 2 patients who didn’t have remission after surgery were treated with steroid therapy. Two patients, who needed to take steroid therapy after surgery and didn’t have remission despite these treatment modalities had radiotherapy. Sixty-six percent (4/6) of patients who were on steroid treatment experienced avascular necrosis. Overall, 55% of patients had radiographic improvement and 45% had stable or deteriorated imaging findings. Endocrinological evaluations revealed that 1 patient (5%) had improvement, 16 patients (85%) had stable findings, 3 patients (15%) had deteriorated endocrinological functioning and 1 patient didn’t have any follow-up data. Six patients who were followed conservatively had no sign of worsening radiologically or endocrinologically.

Conclusion: It is a challenge to make the right diagnosis and also the appropriate treatment of hypophysitis. It is unclear whether active treatment with steroids improves clinical outcome. When the serious side effects of steroids are also taken into account, surgery and or radiotherapy can be appropriate treatment modalities for selected patients.