Xanthomatous Hypophysitis Presenting in an Adolescent Girl:
Primary hypophysitis refers to the isolated inflammation of the pituitary gland not associated with other secondary causes. Among its histopathologic subtypes, xanthomatous is the rarest.

Authors describe a 22-year-old woman with xanthomatous hypophysitis (XH), its clinical progression over 8 years as well as the treatment effects of prednisolone and azathioprine. This patient was first referred for severe short stature and delayed puberty at the age of 14 years.

-- Investigations revealed multiple pituitary deficiencies.

-- Magnetic resonance imaging showed a pituitary mass whereby a partial resection was performed.

-- A full resection was not feasible due to the location of the mass.

-- The histopathologic analysis of the tissue was consistent with XH. The results of secondary workout for neoplasm, infection, autoimmune, and inflammatory disorders were negative.

-- After surgery, a progressive enlargement of the mass was observed.

-- Two courses of prednisolone were administered with a significant reduction in the mass size.

-- Azathioprine was added due to the unsustained effects of prednisolone when tapered off and the concern of steroid toxicity with continued use.

-- No further increase in the mass size was noted after 6 months on azathioprine.

Conclusively, glucocorticoid and immunotherapy are treatment options for XH; however, more cases are needed to better understand its pathogenesis and clinical progression.

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8165125/