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SUMMARY We suspected the presence of pheochromocytoma and were able to conrm our diagnosis in a patient presenting with symp-toms of fatal cardiogenic shock due to catecholamine-induced car-diomyopathy. Furthermore, we evaluated the possibility of MEN 2A as the source of the thyroid nodules and diagnosed MTC using various tests and surgery. The patient had a younger sister who un-derwent follow-up observation for benign thyroid nodules, and as a result, the diagnosis of MTC was established during selective test-ing for MEN 2A. Genetic testing for the RET gene demonstrated that the diagnosis of MEN 2A was due to a mutation of C634R on exon 11. Using a selective test for familial genes, mutations of the same genes were conrmed, and a family of MEN 2A individuals was identied. ACKNOWLEDGEMENT The authors appreciate the efforts of Young Ji Yoon in the prepa-ration of the manuscript. REFERENCES 1. Mulligan LM, Kwok JB, Healey CS, Elsdon MJ, Eng C, Gardner E, LoveDR, Mole SE, Moore JK, Papi L, Ponder MA, Telenius H, Tunnacliffe A, Ponder BJ: Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A. Nature 363:458-460, 1993

A Family Presenting with Catastrophic Features due to Pheochromocytoma Associated with Multiple Endocrine Neoplasia 2A