Bilateral synchronous adrenalectomy for bilateral adrenal pheochromocytoma

Dear Editor Bilateral pheochromocytoma is a rare condition, accounting for 7% to 10% of all pheochromocytomas. It is often associated with hereditary factors, with 60% to 90% harboring germline mutations1. The goal of surgery is to achieve complete tumor resection without rupture, including en bloc resection of adjacent infiltrated organs. Cortical-sparing adrenalectomy is recommended for bilateral pheochromocytoma, as it avoids the need for lifelong corticosteroid replacement therapy, although, high-level evidence supporting it is still limiting2. A 19-year-old male presented with blurring of vision, excessive sweating, and headache for 2 months. Physical evaluation revealed blood pressure of 200/110 mm Hg, heart rate of 114/min, and bilateral papilledema. Biochemical tests showed markedly elevated plasma metanephrines (free metanephrines: 1160 ng/L and free normetanephrines: 15,000 ng/L). Contrast-enhanced computed tomography scan revealed a 72 × 60 × 50 mm mass (+64 HU) arising from the right adrenal gland, which was abutting the liver superiorly and inferior vena cava medially. Similarly, a 30 × 27 × 25 mm mass (+60 HU) was arising from the left adrenal gland. Diagnosis of bilateral pheochromocytoma was made and a synchronous bilateral adrenalectomy was planned. To counteract the hemodynamic changes during the intra- and postoperative periods, alpha blocker (prazosin 1.5 mg twice daily) for 14 days and beta blocker (carvedilol 6.25 mg twice daily) for 3 days before the surgery were prescribed. As per the recommendations, the patient underwent laparoscopic cortical-sparing transperitoneal adrenalectomy in the left side through 3 ports and open adrenalectomy via a right subcostal incision in the right side. The intraoperative period was uneventful. The patient was monitored closely in the high-care unit for 3 days, with strict monitoring for hypotension and hypoglycemia. He was discharged on the sixth postoperative day. The biopsy confirmed bilateral benign pheochromocytoma, with no signs of lymphatic, vascular, or capsular invasion. Microscopic findings showed uniform nuclei and abundant clear cytoplasm arranged in a Zellballen pattern with areas of hemorrhage and necrosis (Fig. 1).Figure 1.: (A) Axial sections of contrast-enhanced CT scan showing 7.25 × 6.08 × 5.03 cm (HU +64) mass arising from the right adrenal gland, abutting liver superiorly and inferior vena cava medially and 3.05 × 2.77 × 2.54 cm (HU +60) mass arising from the left adrenal gland, suggestive of bilateral pheochromocytoma. (B) Postoperative specimen of right and left adrenal gland. (C) Microscopic examination of bilateral adrenal gland showing tumor cells arranged in a nested, trabecular, and solid pattern in low power view (H&E stain X10). CT, computed tomography; H&E, hematoxylin and eosin.The patient is on regular follow-up, with no headache, reduced sweating, and no need for corticosteroid supplementation, 12 months post surgery. The management of bilateral pheochromocytoma requires a multidisciplinary approach with careful preoperative planning, surgical resection, and long-term follow-up. Before surgery, it is crucial to control blood pressure and prevent catecholamine-induced complications, such as arrhythmias. This is typically achieved using alpha-blockers (eg, phenoxybenzamine) followed by beta-blockers to control tachycardia. Adequate hydration is also essential, as catecholamine-induced vasoconstriction can lead to hypovolemia3. In this case, alpha blocker (prazosin 1.5 mg twice daily) for 14 days and beta blocker (Carvedilol 6.25 mg twice daily) for 3 days before the surgery were prescribed. The treatment strategy depends on the size of the tumors, the patient’s overall health, and the underlying genetic syndrome. Surgical resection is particularly challenging in bilateral cases. This patient underwent synchronous left laparoscopic transperitoneal adrenalectomy via 3 ports and right open adrenalectomy via right subcostal incision. We planned for open transperitoneal adrenalectomy in the right side as the tumor size was >6 cm4. Cortical-sparing adrenalectomy for bilateral pheochromocytoma preserves adrenal function, reducing the risk of adrenal insufficiency and the need for lifelong hormone replacement therapy. However, the procedure is technically challenging and carries a risk of incomplete tumor removal and recurrence1. In this case, we did a total adrenalectomy in the right side and a cortical-sparing adrenalectomy for left side. Postoperatively, the focus shifts to managing adrenal insufficiency, especially in patients who undergo bilateral adrenalectomy. In this case, as we had done cortical-sparing adrenalectomy, the patient did not require steroid replacement therapy. Long-term follow-up with periodic imaging and biochemical testing is essential for detecting any recurrent or new tumors3. In this case, it has been 12 months since the operation, and there is no evidence of recurrence or the emergence of new tumors. In conclusion, bilateral pheochromocytoma is rare, with surgical resection as the definitive treatment. Genetic testing is strongly recommended, and vigilant postoperative follow-up is essential. Acknowledgments The authors would like to thank the staff of the operation room and the general ward for providing support and helping in the management of the patient.