Xanthogranulomatous hypophysitis: a rare and often mistaken pituitary lesion

Endocrinology, Diabetes & Metabolism Case Reports
Jaya Sujatha Gopal-KothandapaniPaul J Dimitri

Abstract

Xanthogranulomatous hypophysitis (XGH) is a very rare form of pituitary hypophysitis that may present both clinically and radiologically as a neoplastic lesion. It may either be primary with an autoimmune aetiology and can occur in isolation or as a part of autoimmune systemic disease or secondary as a reactive degenerative response to an epithelial lesion (e.g. craniopharyngioma (CP), Rathke's cleft cyst, germinoma and pituitary adenomas) or as a part of a multiorgan systemic involvement such as tuberculosis, sarcoidosis or granulomatosis. It may also present with a variation of symptoms in children and adults. Our case series compares the paediatric and adult presentations of XGH and the differential diagnoses considered in one child and two adult patients, highlighting the wide spectrum of this condition. Endocrine investigations suggested panhypopituitarism in all three patients and imaging revealed a suprasellar mass compressing the optic chiasm suggestive of CP or Rathke's cleft cyst in one patient and non-functioning pituitary macroadenoma in two patients. Magnetic resonance imaging (MRI) demonstrated mixed signal intensities on T1- and T2-weighted sequences. Following endoscopic transsphenoidal surgery, histological ana...Continue Reading

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Citations

Jan 26, 2017·Endocrine Pathology·Kai DuanOzgur Mete
Jul 3, 2019·Journal of the European Academy of Dermatology and Venereology : JEADV·H Husein-ElAhmedM Steinhoff

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Methods Mentioned

BETA
biopsy
hormone replacement therapy
hormone replacement

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