Case Report: Surgical Intervention Under Pheochromocytoma Multisystem Crisis: Timing and Approach

Abstract

BackgroundProgressive multiple organ failures still occur in some patients with pheochromocytoma multisystem crisis (PMC) despite α- and β-blockade being used, and emergency adrenalectomy may lead to rapid hemodynamic stabilization and recovery. Therefore, the optimal timing and surgical approach under PMC remain controversial.Case PresentationA 50-year-old man presented with persistent chest pain accompanied by vomiting and headache. CT showed a right adrenal mass, and plasma catecholamine levels were significantly elevated. Phenoxybenzamine was used, but his symptoms were aggravated. He progressed to acute respiratory distress syndrome (ARDS) and received mechanical ventilation. Reexamination of CT showed pheochromocytoma rupture. Emergency pheochromocytoma resection was performed on the 5th day, and he was discharged on the 21st day. A 46-year-old woman was admitted for intrauterine device removal and received hysteroscopy under intravenous anesthesia. She presented with dyspnea, fluctuating blood pressure, and loss of consciousness 9 h after hysteroscopy surgery. CT showed a left adrenal mass, and plasma catecholamine levels were significantly elevated. Her condition fluctuated and could not meet the preoperative preparation criteria for pheochromocytoma despite adequate doses of α-blockade and β-blockade were taken. Furthermore, her lung condition worsened due to recurrent crises and pulmonary edema. After multidisciplinary discussions, laparoscopic left adrenalectomy with venoarterial extracorporeal membrane oxygenation (VA-ECMO) support was performed on the 28th day, and she was discharged on the 69th day.ConclusionElective surgical resection is the essential therapy for PMC with adequate preoperative medical management. Emergency surgery is recommended for patients who fail to achieve medical stabilization or progressive organ dysfunction within 1 week, especially those with tumor rupture and uncontrolled bleeding. The laparoscopic approach may represent an option even under PMC.

Pheochromocytoma Multisystem Crisis Complicated by Severe Acute Pancreatitis

“…Since the cardiac function was not deteriorated in the current case, the etiology of the interstitial shadow was not likely cardiogenic. The lung shadow subsided without antibiotics and thus was suggested to be affected by complex vascular and humoral factors due to PMC and acute pancreatitis ( 1 , 3 , 9 ).…”

“…Extreme hyperglycemia and hypertriglyceridemia as metabolic emergencies were partly ameliorated after the improvement of acute pancreatitis and completely improved after adrenalectomy. As a treatment of PMC, appropriate blood pressure control by medications, such as α- or β-blockers, followed by the surgical removal of the adrenal mass is recommended, as was conducted in the current case to prevent intra- and post-operative hypotension ( 9 ).…”