Hereditary syndromes are responsible for a small number of pheochromocytomas. These hereditary pheochromocytomas are frequently bilateral and rarely malignant. Surgical treatment remains controversial secondary to the risk of Addisonian crisis and need for lifelong corticosteroid therapy associated with bilateral total adrenalectomy. Recently it has been reported that cortical sparing bilateral adrenalectomy is safe and can successfully avoid the need for replacement therapy.
We present a case of a 21 year Hispanic male who presented with headaches, palpitations, and severe hypertension and was found to have bilateral pheochromocytomas by biochemical analysis, MIBG, and computed tomography scanning. The patient’s brother had previously undergone a right adrenalectomy and removal of extra adrenal pheochromocytoma. Patient underwent a laparoscopic bilateral cortical sparing adrenalectomy. The technique including pertinent positives, trocar placement, dissection, and method of excision are demonstrated. The patient had no postoperative morbidity and was discharged home on postoperative day 2. Pathology revealed a 23 gm/33 gm right cortical benign pheochromocytomas, both with low Ki67 proliferation indices. Two month follow-up ACTH stimulation test has shown normal 30 min aldosterone and 60 min cortisol stimulation levels. The patient has remained asymptomatic thus far, and has avoided the need for adrenal cortical replacement.
Laparoscopic bilateral cortical sparing adrenalectomy is a safe, technically feasible treatment for hereditary pheochromocytoma. These patients must have long term follow-up including monitoring of the remnant gland for recurrence and yearly biochemical screening studies.
Session: Podium Video Presentation
Program Number: V013