Endocrine Abstracts

We describe a 68 year lady who was found to have bilateral adrenal masses (7 cm on the right side and 1.3 cm on the left) during investigations for gastrointestinal symptoms, in early 2016. Biochemistry revealed high urinary normetanephrines of 41.7pmol/24h (ref 0–3) and metanephrines of 40pmol/24h (ref 0–1.8). A PET and MIBG scan revealed uptake in both adrenals and therefore a diagnosis of bilateral phaeochromocytoma was made. The patient had complained of occasional palpitations and headaches, but no paroxysmal sweating or flushing. She had long-standing borderline hypertension not requiring treatment, and diet-controlled type 2 diabetes. In 1988 she had had a sub-total thyroidectomy for a large goitre at another hospital. The patient had a family history of thyroid disease. Her paternal aunt had a goitre removed and one of her sisters has autoimmune hypothyroidism. Her identical twin also has type 2 diabetes. Her mother had a stroke and osteoarthritis and her father died of a myocardial infarction. She has two daughters aged 38 and 41 years old. The patient underwent bilateral adrenalectomy in August 2016. Subsequent histology confirmed a right sided phaeochromocytoma, with a PASS 9, and a left sided phaeochromocytoma, with a PASS 4. Genetic screening revealed a previously reported ATA level C pathogenic RET variant (p Cys634Tyr)confirming a diagnosis of MEN 2A. This varian is associated with more aggressive form of medullary thyroid carcinoma. In light of these findings an ultrasound scan of her thyroid was performed in September 2016. This revealed two nodules measuring 13mm and 4mm in the right lobe, and a 10mm nodule in the right-sided isthmus. These nodules had entirely benign ultrasound appearances. Calcitonin and carcinoembryonic antigen (CEA) levels were normal. We were able to obtain the histology slides taken from the patients’ previous sub-total thyroidectomy. These had initially been reported, in 1988, as benign multi-nodular changes, but in retrospect a clear focus of medullary thyroid carcinoma was found. The question therefore was raised about whether the patient should now proceed to completion thyroidectomy, despite there being no definitive evidence of malignancy on ultrasound and 30 years having passed since her original thyroid surgery.

What intervention, if any, would you recommend in this situation?

What investigations, if any, would you look to perform prior to making your recommendation?

If you decided against completion thyroidectomy, what monitoring would you put in place?

How would you council the patient and her family?