Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2014) 36 P80 | DOI: 10.1530/endoabs.36.P80

BSPED2014 Poster Presentations (1) (88 abstracts)

Variable presentation of xanthogranulomatous hypophysitis: a case series

Jaya Sujatha Gopal-Kothandapani 1 , Veejay Bagga 2 , Stephen B Wharton 3 , Daniel J Connolly 4 , Saurabh Sinha 2 & Paul Dimitri 5


1Department of Human Metabolism, University of Sheffield, Sheffield, UK; 2Department of Neurosurgery, Royal Hallamshire Hospital and Sheffield Children’s Hospital, Sheffield, UK; 3Department of Histopathology, Royal Hallamshire Hospital, Sheffield, UK; 4Department of Neuroradiology, Royal Hallamshire Hospital and Sheffield Children’s Hospital, Sheffield, UK; 5Department of Paediatric Endocrinology, Sheffield Children’s Hospital, Sheffield, UK.


Introduction: Xanthogranulomatous hypophysitis (XGH) is a very rare form of pituitary hypophysitis that may present both clinically and radiologically as a tumour. Our case series compares paediatric and adult presentations of XGH.

Case series: Patient 1: A 15-year-old female presented with refractory headache, lethargy, short stature, delayed growth (weight (−3.36) SDS, height (−1.73) SDS, BMI 14 kg/m2), and pubertal arrest over 18 months. Formal visual fields demonstrated bitemporal quadrantanopia.

Patient 2: A 21-year-old female presented with lethargy, frontal headaches and secondary amenorrhoea, 3 years after delivery. Postpartum she had normal lactation and menstrual irregularity followed by secondary amenorrhoea. Visual examination was normal.

Patient 3: A 67-year-old man was diagnosed with hypopituitarism following an episode of collapse and intermittent temporal headaches. He had a long standing history of rheumatoid arthritis and rheumatoid nodules within the prostate. Eye examination was normal.

Endocrine investigations demonstrated panhypopituitarism in all three patients. Patient 2 had hyperprolactinaemia requiring Cabergoline.

Pituitary MRI revealed a suprasellar mass compressing the optic chiasm suggestive of craniopharyngioma or rathke’s cleft cyst in patient 1; suspected non-functioning pituitary macroadenoma in patients 2 and 3. MRI demonstrated mixed signal intensities on T1- and T2-weighted sequences.

Following endoscopic trans-sphenoidal surgery, histology revealed areas with cholesterol cleft formation associated with multinucleate giant cells and numerous macrophages in patients 1, 2 and necrobiotic granulomatous chronic inflammatory infiltrate with neutrophils in patient 3. Immunohistochemical staining for IgG4 performed in patient 3 showed IgG4 positive plasma cells (>5%) raising possibility of a systemic IgG4 related process more commonly associated with ocular necrobiotic xanthogranuloma.

Conclusion: This case series describes the spectrum of XGH disease which is yet to be defined. Mixed signal intensities on T1- and T2-weighted MRI sequences may indicate XGH and diagnosis is confirmed by histology. Histological variation may indicate an underlying systemic process.

Volume 36

42nd Meeting of the British Society for Paediatric Endocrinology and Diabetes

British Society for Paediatric Endocrinology and Diabetes 

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