Xantho-Granulomatous Hypophysitis: Analysis Of Clinical, Biochemical, Radiological And Etiological Characteristics

Abstract

Introduction: Xanthogranulomas (XG) are chronic inflammatory lesions caused by accumulation of lipid laden macrophages with granuloma formation, usually secondary to hemorrhage, infarction or necrosis upon existing neoplasm or cyst. Primary sellar xanthogranulomas are rare and may represent a stage in xanthomatous hypophysitis (XH) evolution.Aim: To analyze clinical presentation, hormonal, radiological and histological features of patients diagnosed with pituitary xanthomatous and xhantogranulomatous lesions in a tertiary clinical center. Material and methods: Data collected from electronic medical histories of patients diagnosed with xanthomatous hypophysistis (XH) and xanthogranulomatous (XG) lesions after pituitary surgery at University Clinical Center of Serbia in Belgrade were analyzed in retrospective manner (2015-2021).Results: Cohort included three pediatric and eleven adult patients (9 male and 5 female), average age 14.6±4.7 years in the pediatric, and 55.3±14.4 years in the adult subgroup. At presentation panhypopituitarism was diagnosed in 8 (57.1%) (7 male, 1 female), hyperprolactinemia in 3 (21.4%) patients, polyuria-polydipsia in only one patient (7.1%). Nine patients (64.3%) presented with headache, 5 (37.5%) with neuro-ophthalmic disorders, 3 (21.4%) with neurological symptoms. On magnetic resonance imaging (MRI) lesions were described as cystic in 3 (21.4 %) or predominantly solid in 11 (78.6%) patients. Secondary forms were associated with Ratke's cleft cyst (RCC) in 3 (21.4%) and pituitary adenoma (PA) in 6 (42.8%) cases. “Pure” sellar XG, independent of preexisting lesions, were found in 5 (35.7%) exclusively male patients. Only one patient was diagnosed with XH, majority with pituitary XG.Conclusion: Our cohort was unique for male preponderance and high prevalence of panhypopituitarism in male patients. XG pituitary lesions were more common than XH. PA followed by RCC were the leading causes of secondary XG lesions. Majority of patients presented with predominantly solid lesions on MRI. The most frequent presenting symptoms were headache and hypopituitarism, followed by neuro-ophtalmic deficits. Polyuria-polydipsia and neurological symptoms were rare at presentation.