SAT-321 Bilateral Cortical Sparing Adrenalectomy For Treatment Of Pheochromocytoma In A MEN2-a Mutation Carrier: 19 Year Follow-up

Background: cortical sparing adrenalectomy is rarely proposed for the treatment of bilateral pheochromocytomas. We describe the longest reported follow-up of this approach in a MEN2-A mutation carrier woman. Clinical case: a 27-year-old woman was referred with a 2-year history of hypertensive crisis, headache, tachycardia, diaphoresis, hot flushes, dyspnea, chest pain, weight loss, and polyuria. Pheochromocytoma was suspected and abdominal CT scan showed a 6 x 5 cm heterogeneous expansive lesion with a central hypodense area in the right adrenal and the left adrenal was lobulated and slightly increased in size, showing a heterogeneous contrast enhancement. 24h-urinary fractionated catecholamines were: norepinephrine 1920 ng/min (n<50 ng/min), epinephrine 386.7 ng/min (n<10 ng/min) and dopamine 1740 ng/min (n<400 ng/min), and the diagnosis of pheocromocytoma was confirmed. She had also medullary thyroid carcinoma, primary hyperparathyroidism and cutaneous lichen amyloidosis and genetic testing for MEN2A has found a C634R mutation in exon 11 of RET proto-oncogene. A bilateral cortical sparing adrenalectomy was proposed aiming to preserve cortical adrenal function. The patient was successfully operated on October/1999 by an open anterior approach. On the first postoperative day her serum cortisol levels were normal. During the post-operative period she developed a surgical site infection and an extensive nosocomial pneumonia that were treated with antibiotics, with no need for gluco- or mineralocorticoid replacement.&nbsp; Subsequently, the patient was regularly followed-up, was asymptomatic, but with sustained hypertension, successfully treated with enalapril monotherapy. For eleven years she had normal urinary metanephrines and abdominal CT scans without signs of pheocromocytoma recurrence and had a successful pregnancy in 2005. In 2010, urinary normetanephrines increased (1.2-1.5 times the upper limit of normal), and the abdominal CT revealed bilateral nodules in the residual adrenals. After discussing therapeutic options with the patient, a decision to maintain clinical follow-up was made. From 2010 until 2015 urinary metanephrines were back to normal range. In 2016, urinary normetanephrines increased again (1.99 times the upper limit of normal), results that were confirmed thereafter, ranging from 2.94 – 4.31 times the normal range. The patient had a transient ischemic attack due to right vertebral artery dissection in 2014. Current abdominal CT reveals bilateral enlargement of the previously described nodules, which has made our team to propose a new bilateral adrenalectomy. Conclusion: this case illustrates the feasibility of cortical sparing surgery in the long-term preservation of adrenocortical function, avoiding the potential risks associated with adrenal insufficiency, and could be considered as a first-line treatment approach.