Takotsubo Cardiomyopathy as the Initial Manifestation of Functional Retroperitoneal Paraganglioma: A Rare and Challenging Diagnosis

Abstract

Background: Paragangliomas are rare catecholamine-secreting neuroendocrine tumors that can cause severe cardiovascular manifestations, including the uncommon presentation of Takotsubo cardiomyopathy mimicking acute coronary syndrome. Aims: To report a rare case of retroperitoneal paraganglioma revealed by catecholamine-induced Takotsubo cardiomyopathy and labile hypertension, highlighting its diagnostic and therapeutic challenges. Presentation of Case: We report the case of a 39-year-old woman who presented with angina-like chest pain associated with labile blood pressure. Further investigations revealed a retroperitoneal paraganglioma complicated by catecholamine-induced Takotsubo cardiomyopathy. Paragangliomas and pheochromocytomas are rare neuroendocrine tumors characterized by excessive catecholamine secretion, which determines their clinical presentation and may lead to severe cardiovascular complications, including resistant hypertension and stress-induced cardiomyopathy. The diagnosis relies primarily on biochemical evidence of catecholamine excess, followed by imaging studies for tumor localization and characterization. Discussion: Excess catecholamine secretion from paragangliomas can result in a wide spectrum of cardiovascular manifestations, making diagnosis challenging, particularly in atypical presentations. Takotsubo cardiomyopathy is a recognized but uncommon complication of these tumors. Early recognition through biochemical and imaging evaluation is essential to establish the diagnosis and prevent potentially life-threatening complications. Preoperative medical optimization with alpha-adrenergic blockade is crucial to achieve adequate blood pressure control before definitive treatment. Conclusion: Surgical resection remains the cornerstone of treatment for paragangliomas and requires appropriate preoperative preparation with alpha-blockers to reduce perioperative risk. This case highlights the importance of considering catecholamine-secreting neuroendocrine tumors in patients presenting with unexplained Takotsubo cardiomyopathy and labile hypertension, and underscores the value of a multidisciplinary diagnostic and therapeutic approach.