Background: Familial pheochromocytoma, increasingly diagnosed in asymptomatic subjects with inherited syndromes such as multiple endocrine neoplasia type 2 and Von Hippel-Lindau disease, is frequently bilateral and multifocal, but very rarely malignant. Therefore, bilateral adrenalectomy and subsequent lifelong steroid replacement, with its attendant side effects, is not desirable. Minimally invasive adrenal sparing surgery by means of laparoscopy was explored for the treatment of bilateral pheochromocytoma. We report on the cure rate and adrenocortical function in a series of patients treated accordingly.
Patients and methods: Four patients (three male, one female, ages 9-60 yr) with hereditary bilateral adrenal pheochromocytoma were treated by laparoscopic surgery in an organ sparing fashion. Postoperatively, all patients were reevaluated for complete tumor removal and for adrenocortical function.
Results: Two to 24 months after surgery, all patients were normotensive, had normal sodium potassium, glucose, aldosterone, renin, and cortisol serum concentrations, 24-h excretion of norepinephrine, epinephrine, and vanillylmandelic acid. Abdominal magnetic resonance imaging (n = 3) and computed tomographic scan (n = 1) disclosed no remnant or relapsing tumor tissue. ACTH stimulation testing resulted in normal cortisol responses.
Conclusion: By adrenal sparing laparoscopic surgery not only bilateral pheochromocytoma can be successfully removed, but also adrenocortical function preserved. This may become the treatment of choice for familial pheochromocytoma.