Granulomatous hypophysitis: experience with eight surgical cases of a single center

Abstract

Background: Although primary hypophysitis is not uncommon, a granulomatous etiology of hypophysitis is relatively rare. Radiological and diagnostic characteristics of several isolated cases have been described to date. However, to the best of our knowledge, surgical treatment of eight cases of granulomatous hypophysitis confirmed by histopathology has not yet been reported. Methods: A retrospective study was performed to review cases of granulomatous hypophysitis from November 2003 to November 2013 in our neurosurgical department. Only eight out of 1345 operations were diagnosed with granulomatous hypophysitis. Results: The cohort included two men and six women. The most common manifestations reported were headache, fatigue and polyuria. All six female patients presented with either menstrual disorders or amenorrhea. Seven of eight patients suffered polyuria. Two patients suffered bitemporal hemianopsia. One was accompanied with eosinophilic granuloma of the skull. Prolactin levels were increased in six patients, four of them were noted to have thyroid axis imbalances, one was noted to be deficient in the gonadotropin axis and two were deficient in the adrenal axis. The lesion demonstrated homogenous enhancement and an abnormally thickened pituitary stalk. A histological diagnosis of granulomatous hypophysitis was established in all eight patients, who were treated surgically. Characteristic granulomas formed by epithelioid histiocytes and multinucleated giant cells were observed. Conclusion: Pure glucocorticoid therapy is less effective than surgical intervention and has not been recommended for the treatment of granulomatous hypophysitis. Although replacement therapy can relieve associated symptoms, we conclude surgical intervention, particularly minimally invasive surgery via a transsphenoidal approach, to be mandatory in establishing a diagnosis and reducing the size of the inflammatory mass. Adjuvant corticosteroids therapy is recommended.

Primary Granulomatous Hypophysitis Presenting with Secondary Hypothyroidism

“…It is believed to be either idiopathic or associated with other autoimmune disorders. Hypophysitis can be categorized histopathologically as LH, GH, xanthomatous hypophysitis, and plasmocytic hypophysitis, also known as IgG4-related hypophysitis (2,3). Mixed histology can be seen, however.…”

“…It causes pituitary enlargement that mimics a macroadenoma (6). Secondary GH can be due to an infection, systemic inflammatory disorders such as sarcoidosis, Wegener granulomatosis, Crohn disease, Takayasu arteritis, Cogan syndrome, or a side effect of some medications, mostly in those receiving immunotherapy for metastatic melanoma with anti-CTLA4 and anti-PDi antibodies (3,5,8). Our patient's rheumatology evaluations, including DNA antibody, SSA/SSB antibodies, anti-Smith/RNP antibody, JO-1 antibody, anti-nuclear antibody, and rheumatoid factor, were within normal limits.…”

“…Nausea and vomiting occur secondary to increased intracranial pressure and/or acute adrenal insufficiency. In such cases, emergency intervention is needed, with treatment of the mass or surgical intervention to relieve the pressure (3,7,9). Hyperprolactinemia can be seen with development of amenorrhea and galactorrhea.…”

“…High-dose glucocorticoids help with symptoms such as headaches by reducing inflammation and thus decreasing the size of the pituitary mass (3,12). Dopamine agonists such as bromocriptine or cabergoline have been used in patients suffering from visual field deficits and hyperprolactinemia.…”

“…It can be idiopathic or manifest as a part of a systemic autoimmune disease. Granulomatous hypophysitis (GH) and lymphocytic hypophysitis (LH) are the most common subtypes reported in literature (1)(2)(3). We present a case of a 45-year-old female with past medical history of hypothyroidism after total thyroidectomy for Graves disease and surgical history of hysterectomy.…”