An adrenal adenoma co-secreting cortisol and aldosterone associated with both a bronchial adenocarcinoma and a papillary thyroid carcinoma. (#237)
A 46 year old woman with a left upper-lobe bronchial adenocarcinoma was referred for evaluation of adrenal masses and a right-sided thyroid mass identified incidentally during staging of her bronchial lesion via PET scan. Abdominal CT identified the adrenal masses: 1.3 x 1 x 1 cm ( 15 Hounsfield units) and 2.5 x 1.5 x 1.5 cm (40 Hounsfield units) in the left and right adrenals, respectively. The right adrenal mass was PET positive.
Born in China, she had a significant family history of malignancy, atypical of any cancer syndrome. Further, she has an 8 year history of treatment-resistant hypertension (despite being on 5 anti-hypertensive agents) associated with hypokalemia. Although not overtly Cushingoid, she had difficulty sleeping and weight gain. Biochemical assessment revealed elevated aldosterone with suppressed renin levels, elevated urinary and salivary cortisol levels, and suppressed ACTH levels. Cortisol levels failed to suppress on 2 day low and high dose oral dexamethasone suppression tests. Thyroid ultrasound with FNA was suspicious for malignancy.
Post-lobectomy, she underwent a unilateral right-sided adrenalectomy, revealing 2 adenomas - 22mm and 7mm, respectively. Post-adrenalectomy, both the hyperaldosteronism and the hypercortisolism resolved, markedly improving her blood pressure. However, she required glucocorticoid replacement and remained persistently hypokalemic. Nine months post-adrenalectomy, she still requires glucocorticoid and potassium replacement. Her blood pressure is well cotrolled on 3 antihypertensives.
She subsequently underwent a hemithyroidectomy, revealing a localised 9 mm BRAF mutation-positive papillary thyroid carcinoma.
This is the first documented case of bilateral biochemically-active adrenal adenomas associated with papillary thyroid, and bronchial carcinomas. Although recognised, adrenal adenomas co-secreting cortisol and aldosterone are relatively uncommon. The basis of the on-going features of mineralocorticoid excess is currently being investigated. This unique cluster of uncommon tumours coupled with her family history, suggests a novel genetic cancer syndrome. The possibility of BRAF mutation expression in the other tumours is being explored.