BACKGROUND: Multiple endocrine neoplasia type 2A (MEN 2A) is a rare autosomal-dominant genetic syndrome characterized by mutations in the RET gene and tumors of endocrine glands, including pheochromocytomas. Pheochromocytoma is a benign primary endocrine tumor of the adrenal glands that produces catecholamines and in rare cases is complicated by pheochromocytoma multisystem crisis (PMC), a life-threatening endocrine emergency caused by severe catecholamine overproduction. This report describes a 42-year-old woman presenting with PMC associated with bilateral pheochromocytomas and MEN2A.

CASE REPORT: The patient presented with a sudden onset of back pain and dyspnea with severe shock. An enhanced computed tomography examination revealed bilateral adrenal gland tumors, and she was diagnosed with PMC complicated by multiple organ failure and catecholamine-induced cardiomyopathy. We introduced simultaneous veno-venous extracorporeal membrane oxygenation and continuous hemodiafiltration. A definitive operation for PMC was performed on hospital day 12, with the intent of complete resection of bilateral pheochromocytomas. The patient was weaned from continuous hemodiafiltration on hospital day 21st day and was finally weaned from artificial ventilation on hospital day 30. Genetic examination revealed a pathogenic RET mutation (Cys634Arg, C634R), leading to the diagnosis of MEN2A. She is now undergoing rehabilitation, with intensive surveillance for thyroid medullary carcinoma and hyperparathyroidism.

CONCLUSIONS: PMC can easily lead to multiple organ failure within a few days. A multidisciplinary approach to PMC can rescue these patients by making surgical pheochromocytoma resection possible. If synchronous bilateral pheochromocytomas are detected, MEN2A should be suspected.

Keywords: Adrenal Gland Neoplasms, Case Reports, crisis, Endocrinology, Multiple Endocrine Neoplasia Type 2a, Pheochromocytoma