Background: Patients with hereditary pheochromocytoma are at risk of the development of bilateral disease. Partial adrenalectomy can preserve adrenal function to avoid the morbidity associated with medical adrenal replacement. Here, we report a multimedia case study of synchronous bilateral partial adrenalectomy by the laparoscopic approach.
Methods: A 13-year-old patient with von Hippel-Lindau disease was found to have high urinary metanephrines and normetanephrines. Computed tomography showed bilateral adrenal tumors (2.5 cm on the right side and 0.9 cm on the left). MIBG scan showed positive uptake in the right adrenal gland without extra-adrenal uptake. After adequate adrenergic blockade, the patient underwent laparoscopic partial adrenalectomy bilaterally.
Results: The left side was approached first with the patient in the right decubitus position. Intraoperative ultrasound was performed to determine the line of tumor excision, which was carefully planned to preserve most of the normal-appearing gland. Both tumors were excised completely with good hemostasis. The main adrenal veins of both sides were precisely preserved. Operative time was 228 minutes. No clinically important hemodynamic fluctuations were noted. Pathologic examination confirmed bilateral entirely excised pheochromocytomas. The patient has not required exogenous corticosteroid replacement at follow-up.
Conclusion: Laparoscopic partial adrenalectomy for bilateral pheochromocytomas is safe and technically feasible. It should be considered the treatment of choice for hereditary pheochromocytoma.