Robotic Bilateral Cortical-Preserving Adrenalectomy in an MEN2A Patient with Steroid Allergy

Background:

There are controversies in advocating cortical-sparing adrenalectomy for bilateral pheochromocytomas associated with multiple endocrine neoplasia 2A (MEN2A). We present a patient with MEN2A and steroid allergies who underwent bilateral robotic posterior adrenalectomy, with cortical-preserving on the left.

Methods:

A 31-year-old lady was found to have bilateral adrenal masses on an ultrasound performed for hematuria. Although she did not have a history of hypertension, biochemical testing showed fourfold elevated plasma and urinary metanephrine levels consistent with bilateral pheochromocytomas. Her thyroid ultrasound showed a 1.6 cm left thyroid nodule, which on biopsy was positive for medullary thyroid cancer. Work-up for primary hyperparathyroidism was negative. MIBG scan showed uptake involving both adrenal glands (4.7 cm right and 1.8 cm left). She was started on phenoxybenzamine in preparation for bilateral adrenalectomy. The patient had an allergy to steroids in the past, and when she was skin tested by the Allergy Department, she was found to have a delayed reaction to type A steroids that include hydrocortisone and fludrocortisone. She was not allergic to type C steroids, which included dexamethasone. Based on her presentation and history, a plan was made for cortical-preserving adrenalectomy on one side to be able to maintain her steroid needs with dexamethasone only postoperatively.

Results:

After keeping her on phenoxybenzamine for 3 weeks, she was brought to the operating room. She was placed on a prone position and initially, right adrenalectomy was performed through a posterior retroperitoneal (PR) approach robotically. This was performed through four trocars and was also guided by intraoperative ultrasound and indocyanine green imaging. Subsequently, the left side was dissected. A cortical-sparing adrenalectomy was performed on the left side by using phrenic vessels for inflow and outflow. The viability of the remnant was confirmed with indocyanine green fluorescence. The procedure lasted 3 hours and 57 minutes, and she was discharged home uneventfully on postoperative day 1 with oral dexamethasone only. At 3 weeks, her plasma metanephrines, aldosterone, plasma renin activity, and electrolytes were normal. At 9-month follow-up, she has been recurrence-free and mineralocorticoid-independent.

Conclusions:

To our knowledge, this is the first report of steroid allergy influencing the surgical strategy in a patient with bilateral pheochromocytoma and MEN2A. Her steroid allergy, rather than traditional teaching, dictated the surgical strategy, which has been efficient in treating her disease. The robot and indocyanine green imaging were useful to be able to expeditiously perform her cortical-preserving bilateral adrenalectomy through a PR approach.

No competing financial interests exist.

Runtime of video: 10 mins 40 secs

This case was presented without the procedural video at the “AAES Interesting Case Session” on April 10–12, 2016, in Baltimore, MD.