Endocrine Abstracts

Introduction: Pheochromocytoma is a rare neuroendocrine tumour, commonly diagnosed between ages 40 and 50, typically presents with hypertension, palpitations, and episodic headache. We would like to report a patient diagnosed through an uncommon presentation -cardiomyopathy.

Case report: A 49-year-old female with hypertension, T2DM and obesity, presented to the emergency department with shortness of breath. Although her CXR & ECG were normal and CTPA excluded PE although showed an incidental lung nodule. Her echocardiogram showed global severe systolic dysfunction, Ejection Fraction 30%, with normal valves. The CT coronary angiogram showed normal coronary arteries and lung function tests also were normal. Interval chest CT scan for the lung nodule revealed right adrenal incidentaloma (4.5 x 2.6 cm heterogeneous attenuation (part-cystic part-soft tissue density, no fat). During the endocrinology consultation, she was noted to have a year history of frequent palpitations, sweating, and dizzy spells. Adrenal investigations revealed normal 1 mg overnight dexamethasone suppression test. Her plasma Normetadrenaline level was 8350 pmol/l (120 - 1180), and Metadrenaline 1250 pmol/l (80 - 510). Repeated metanephrines yielded similar results. Confirming pheochromocytoma. Dedicated adrenal MRI showed right-sided 4cm heterogenous cystic adrenal lesion, lacking signal drop-out on out-of-phase, making adenoma unlikely. Interestingly, MIBG scan didn’t show any abnormal uptake. The patient underwent uneventful laparoscopic right adrenalectomy, which confirmed histologically benign pheochromocytoma, PASS score of 5/20 and low level of SDHB expression. Patient also had incidental U2 thyroid nodule – however calcitonin levels came back normal and genetic testing for MEN was negative. Her 6-months postoperative echocardiogram showed recovery from cardiomyopathy (EF 55-59%), and cardiac medications were withdrawn.

Conclusion: Thorough history taking, and careful investigations are crucial in diagnosing the underlying cause of unexplained cardiomyopathy. Although uncommon, recognizing Takotsubo cardiomyopathy in pheochromocytoma is vital for improving the clinical outcomes.