Thrombotic Microangiopathy After Spontaneous Pheochromocytoma Rupture: A Rare MEN 2A Case

Abstract

Introduction Tumors that originate from chromaffin cells in the adrenal medulla and secrete catecholamine are called pheochromocytoma (1). Catecholamine producing tumors are rare, and their incidence is 2 to 8 cases/million people yearly (2). This disease commonly affects people in their 40s and 50s, but it occurs earlier in people with diseaseassociated germline mutations. Although these tumors are typically sporadic, they are also associated with genetic disorders, including multiple endocrine neoplasia syndrome type 2 (MEN 2). Their symptoms may occur in episodes or paroxysmally and involves a broad clinical spectrum due to the high circulating catecholamine level in plasma. Typical symptoms are tachycardia, pallor, headache, and sweating (3). Hypertension occurs in apPheochromocytoma is an adrenal medulla-derived tumor originating from the chromaffin cells that produce and secrete catecholamines. These tumors usually occur sporadically, but they may also be associated with genetic diseases, such as multiple endocrine neoplasia syndrome type 2 (MEN 2). A hypertensive crisis that occurs after the spontaneous rupture of pheochromocytoma, is a rare clinical complication with a high mortality rate. In this article, we present a male case who developed hypertensive crisis and thrombotic microangiopathy (TMA) after a spontaneous pheochromocytoma rupture due to MEN 2A.