Category: Pituitary Disorders/Neuroendocrinology

Monitor: 2

2 - XANTHOGRANULOMATOUS HYPOPHYSITIS: REPORT OF 2 CASES WITH DIFFERENT CLINICOPATHOLOGIC PRESENTATIONS

Saturday, Apr 27
10:30 AM – 11:00 AM

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.


Methods :

Report 2 cases of XGH with different presentations.


Results :

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.


 


Discussion :

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.

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Janelle Violago

Fellow
Allegeny Health Network Endocrinology Fellowship Program
Pittsburgh, Pennsylvania

First year fellow in Endocrinology

Kymberly Gyure

Vice Chair and Director of Anatomic Pathology
Allegheny General Hospital

Is a Neuropathologist in Allegheny General Hospital

Saira Khan

Assistant Program Director
Allegheny Health Network Endocrinology Fellowship Program

Dr Khan is currently the Assistant Program director of AHN Endocrinology Fellowship program

Murray Gordon

Key Clinical Faculty
Allegheny Health Network Endocrinology Fellowship Program

Dr Gordon is an active Faculty member of AHN Endocrinology fellowship program