Central Hypogonadism/Hypothyroidism with a possible Cortisol secreting Adrenal Adenoma in a Patient with Light Chain Systemic Amyloidosis

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 1-36-Adrenal Incidentaloma & Carcinoma
Clinical
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-28
Alan Scott Sacerdote*1, Gul Bahtiyar2, Krishna Patibandla3, Anju Paul4 and Nithin Georgey Thomas3
1Woodhull Med & Mental Hlth Ctr, Brooklyn, NY, 2Woodhull Medical Center, Brooklyn, NY, 3Woodhull medical center, Brooklyn, NY, 4New York University, New York, NY
The endocrine system is frequently involved in systemic amyloidosis [SA]. SA may be associated with light chain, serum amyloid A or transthyretin deposition most commonly, although at least 25 proteins, all of which stain with congo red dye have been reported. Localized amyloidosis [LA] of the pancreatic islets and of the pituitary has been reported in association with aging, type 2 diabetes and Alzheimer’s disease respectively, this LA should not be confused with SA. Pituitary amyloid infiltration is rare in SA, due to relative impermeability of the blood-brain barrier to amyloid protein, and is even more rarely associated with endocrine dysfunction. 15 % of adrenal incidentalomas could be functional namely pheochromocytoma, primary hyperaldosternosim or cortisol secreting. While amyloid adrenal infiltration has been reported in SA, SA with a possible cortisol producing adrenal adenoma has not been reported.

Our patient is a 50-year-old post-menopausal woman with hypertension, dyslipidemia who was admitted to hospital for exertional dyspnea, likely due to restrictive cardiomyopathy thought to be caused by her SA (light chain type). Endocrine consult was done as her abdominal CT revealed a left adrenal mass measuring 4.5×5.4×4.5 cm. Her blood pressure was 100/65 mm Hg. 24 hour urine catecholamine level and serum aldosterone level were normal ruling out pheochromocytoma and primary hyperaldosteronism. 24-hour UFC by liquid chromatography-mass spectrometry was 99.3 mcg/24 hours (4.0-50.0) with 24-hour creatinine 1.45 g/24hours (0.63- 2.50). After 1 mg dexamethasone suppression test, 8 a.m. cortisol level by immunoassay [IA] was 5.1 mcg/dl (2-10 equivocal) and dexamethasone level by extraction chromatography, IA was 39 ng/dl (180-550). The low dexamethasone level could be due to amyloidosis of GI tract causing malabsorption. Plasma ACTH by IA was <5 pg/ml (5-27). On further work up serum TSH by chemiluminescence was 0.19 mIU/L (0.40- 5.50), total T3 by IA was 35.34 ng/dl (60- 181), total T4 by IA was 3.2 mcg/dl (4.5-12.0), T3RU by spectrophotometry was 57.14% (24-40), FSH by IA was 0.99 mIU/ml (23.0-116). LH by IA was 0.07mIU/ml (10.0- 54.7). Reverse T3 by IA was 29 ng/dl (11-32) ruling out euthyroid sick syndrome. The patient is also being investigated for a myeloproliferative disorder due to platelet count of 1.3 million/ul.

In summary, this patient illustrates a presentation of SA with central hypothyroidism/ hypogonadism, which is extremely rare, and a possible cortisol secreting adrenal adenoma, which has not been reported previously.

Nothing to Disclose: ASS, GB, KP, AP, NGT

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