Category: Other - Multiple Endocrine Neoplasia

Type: e-Poster

Monitor: 33

33 - ADRENAL ADENOMA MASKING MULTIPLE ENDOCRINE NEOPLASIA 2A

Friday, Apr 26
11:00 AM – 11:30 AM

Objective : Raise awareness, of the discrete presentation, of an otherwise typically healthy female masking multiple endocrine neoplasias and review the work-up for occult concomittent conditions, treatment options and follow up management.


Methods : We present the biochemical and diagnostic workup of an adrenal adenoma greater than 4cm which masked a compilation of multiple endocrine neoplasias(MEN), discuss management options, and surveillance.


Results :

47year old female presented to Orlando VA Endocrine Clinic in 2017 for evaluation of 4.1cm right adrenal mass found incidentally on GI work-up at non-VA facility in 2013. Patient had mass previously biopsied without biochemical workup and was informed biopsy results were benign. In light of risk of malignancy and consideration for repeat biopsy or surgery, a biochemical workup was done showing co-secretion of hypercortisolism and catecholamines. Serum metanephrines and normetanephrines were greater than twice the upper limit of normal along with elevated urine epinephrine, and  dexamethasone suppression test did not suppress. An otherwise normotensive asymptomatic patient underwent right laparoscopic adrenalectomy. Post-operatively patient a dexamethasone suppression test was administered in clinic which demonstrated retained left adrenal function. Surgical pathology report returned synaptophysin and chromogranin A positive pheochromocytoma with focally positive margins. Given a significant family history of thyroid cancer, hypertension, and suspected adrenal disease, evaluation for ret-proto oncogene was done and returned positive. Patient was subsequently screened for MEN2A. Calcitonin level returned elevated and parathyroid was within normal limits. Patient was recommended to undergo a prophylactic thyroidectomy for risk of medullary thyroid carcinoma which returned positive for bilateral disease with the largest measuring 0.4x0.4x0.3cm on the left lobe. MEN2A genetic testing returned positive and family was also recommended to consider testing as well. Follow up of patient revealed normalization of metanephrines, cortisol, and calcitonin.


Discussion : Multiple endocrine neoplasias may be obscured by the low levels of hormones they produce, however, a low threshold of suspicion may prevent metastatic disease and improve morbidity and mortality. 




Conclusion : Although patient may not have the typical triad of a pheochromocytoma or the insulin requirements of a morbidly obese diabetic, adrenal workup of co-secreting hormones for adrenal adenomas are indicated prior to biopsy, medical management, or surgery and there should be low threshold of suspicion for evaluation of concurrent endocrinopathies.

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Daise Vieira

Endocrinology Fellow
University of Central Florida College of Medicine
Orlando, Florida

Endocrinology Fellow at the University of Central Florida College of Medicine.

Arelys Ramos

Endocrinology Fellow
University of Central Florida College of Medicine
Orlando, Florida

Endocrinology Fellow at UCF COM

Suzanne Martinez

Program Director
Orlando VA Hospital
ORLANDO, Florida

DR. MARTINEZ IS PROGRAM DIRECTOR FOR THE HCA, UNIVERSITY OF CENTRAL FLORIDA CONSORTIUM ENDOCRINOLOGY, DIABETES AND METABOLISM FELLOWSHIP AT THE ORLANDO VETERANS HOSPITAL.

Daise Vieira

Endocrinology Fellow
University of Central Florida College of Medicine
Orlando, Florida

Endocrinology Fellow at the University of Central Florida College of Medicine.