Necrotizing Infundibuloneurohypophysitis: Case Report and Literature Review

Abstract

Several conditions may lead to pituitary necrosis: Sheehan syndrome, increased intracranial pressure, traumatic pituitary injury, thrombosis of the vessels perfusing the pituitary, ischemia, as well as various infections and autoimmune processes. Necrosis may develop in the anterior and posterior lobes, as well as in the pituitary stalk and infundibulum. Hypophysitis is a chronic inflammatory disorder resulting from an autoimmune process (primary hypophysitis) [1, 2] or from an identified agent, such as infection (secondary hypophysitis) [3]. By histology, in various forms of hypophysitis, necrotic areas may be apparent. Necrosis is usually small and focal in primary hypophysitis [4]. To date, to our knowledge, there are only a few published cases where necrosis occupied large portions of the hypophysis [4, 5]. We report the case of a 13-year-old female with central diabetes insipidus (DI) and hypopituitarism. Magnetic resonance imaging (MRI) showed homogeneous gadolinium enhancement in the upper pituitary stalk and on the floor of the third ventricle. The lesion was surgically removed. The histologic, immunohistochemical, and electron microscopic examinations of the surgical specimen revealed necrotizing infundibuloneurohypophysitis.