Category: Pituitary Disorders/Neuroendocrinology
Objective : Xanthogranulomatous hypophysitis (XGH) is the rarest subtype of pituitary hypophysitis characterized by the presence of cholesterol clefts, multinucleated giant cells,inflammatory infitrates, hemosiderin and fibrosis. Pathogenesis,clinical and radiologic features and disease course are not well defined.
Report 2 cases of XGH with different presentations.
1: 42M w/ T2DM and Hashimoto’s hypothyroidism on LT4 presented w/ headache,decreased libido and early morning erections.ACTH 14 (10-48pg/ml),AM cortisol 3.9 (6-18.4mcg/dL) c/w secondary AI, growth hormone deficiency (GHD):IGF-1 32 (43-203ng/ml), secondary hypogonadism: total 17 (264-916ng/dL),free testosterone 2.8 (40-250ng/dL) w/ LH 0.4 (2-12mIU/ml) and FSH 1 (1-12mIU/ml), concomitant central with primary hypothyroidism: FT4 0.82 (0.7-1.9ng/dL),TSH 1.77 (0.4-4mcU/ml).MRI showed pituitary mass 21x22x22 mm T1 bright,T2 heterogenous w/ peripheral enhancement c/w pituitary apoplexy.Visual fields( VF) negative.He underwent transphenoidal resection (TSR) w/ pathology:extensively necrotic pituitary adenoma w/ xanthoma cells, hemosiderin,and cholesterol clefts. Headache improved postop.Pituitary functions w/ GST showed persistent panhypopituitarism,rx with hydrocortisone, LT4, testosterone and GH.
2: 41F presented w/ polyuria,polydipsia,fatigue,cold intolerance,amenorrhea, and galactorrhea.Work up c/w DI:serum Na 143 (134-142mmol/L),urine osmolality 76 mOsm/kg, central hypothyroidism:FT4 0.6 ng/dl,TSH 2.95 mcU/ml, hypogonadism:estradiol 34 (64-357pg/ml) LH <0.2 FSH 1.2,high prolactin 72.4 (3-30ng/ml).Normal ACTH stim test and IGF-1.MRI showed enhancing mass 18x11x16 mm,abuts optic chiasm,enlarged pituitary stalk .VF negative.Rx w/ DDAVP and LT4.Pathology after TSR:xanthogranulomatous inflammation w/ lymphocytes,plasma cells,cholesterol clefts and foreign body giant cell.Secondary hypogonadism, hypothyroidism persist,galactorrhea resolved.
XGH may be primary autoimmune,secondary inflammatory reaction or part of systemic granulomatous disease.The different pathologies of the 2 cases point to different etiology-the first is a secondary reaction to pituitary adenoma apoplexy;second:a primary autoimmune lesion.Headache,visual disturbance and galactorrhea due to mass effect and hypopituitarism from infiltration of both lobes are common presentations.Both had hypopituitarism,the second w/ associated DI.Local mass effect presented as headache and hyperprolactinemia.MRI shows sellar mass or cyst. Prognosis is favorable after TSR w/ only 1 reported recurrence.Local mass effect resolves postop but hypopituitarism persists as shown in both cases.
Conclusion : XGH can have different pathogenesis and clinical presentations.
Janelle Violago– Fellow, Allegeny Health Network Endocrinology Fellowship Program, Pittsburgh, Pennsylvania
Kymberly Gyure– Vice Chair and Director of Anatomic Pathology, Allegheny General Hospital
Saira Khan– Assistant Program Director, Allegheny Health Network Endocrinology Fellowship Program
Murray Gordon– Key Clinical Faculty, Allegheny Health Network Endocrinology Fellowship Program
Allegeny Health Network Endocrinology Fellowship Program
First year fellow in Endocrinology
Vice Chair and Director of Anatomic Pathology
Allegheny General Hospital
Is a Neuropathologist in Allegheny General Hospital
Assistant Program Director
Allegheny Health Network Endocrinology Fellowship Program
Dr Khan is currently the Assistant Program director of AHN Endocrinology Fellowship program