Endocrine Abstracts

Introduction: Pheochromocytoma is a rare catecholamine secreting neuroendocrine tumor. We describe a rare case of Tako – Tsubo cardiomyopathy as first manifestation of pheochromocytoma, which later developed into PMC.

Case report: A 39-year-old woman, with no history of arterial hypertension, was admitted to the emergency room with acute headache, severe chest pain radiating to the back, nausea and vomiting. At presentation, patient was in cardiorespiratory distress, tachycardic, tachypneic, with signs of pulmonary edema. Electrocardiogram showed supraventricular tachycardia with minor ST-segment depressions at V₅ through V₆ leads. Transthoracic echocardiography revealed akinesis of the middle segments of the left ventricle (LV), excessive apical and basal LV segments contractions and significantly impaired LV ejection fraction of 35–40%. Blood test analysis showed elevated troponin, significantly elevated brain natriuretic peptide (BNP), metabolic acidosis, together with increased hepatic enzymes. Chest and abdominal CT scan was performed and a massive left adrenal mass of 107 × 93 × 137 mm was found, so catecholamine – induced stress related (Tako-Tsubo) cardiomyopathy was highly suspected. Blood samples for plasma metanefrin and normetanefrin were taken to confirm the diagnosis of pheochromocytoma. During first 24-hours sepsis, acute renal failure and hypotension developed, thus intravenous infusion of vasopressors was started. However, patient’s hemodynamics became unstable, cardiac function deteriorated – troponin I and BNP increased, hypotension and tachycardia remained despite maximal doses of vasopressors. Intra-aortic balloon pump was implanted and α-blocker labetolol 20 mg per hour intravenously and ß-blocker metoprolol 25 mg orally was started. Hemodynamics was stabilized and oral phenoxybenzamine 10 mg bid was prescribed. On day 9th the intra-aortic balloon pump was removed, the dose of phenoxybenzamine was increased to 40 mg per day. Laboratory analyses showed improvement in renal and liver, echocardiography showed transient previously noted wall motion abnormalities with a normal ejection fraction of > 55%. On the 15th day of hospitalization, patient underwent an exploratory laparoscopy, due to bleeding, turning into a laparotomy and excision of the large left adrenal mass. The pathological analysisand elevated plasma metanefrin and normetanefrin confirmed pheochromocytoma. After operation, patient was stable, her vitals were normal, renal, liver and heart function normalized. During follow-up, serum metanephrines were normal. Also abdomen CT scan showed no tumor recurrence.

Conclusion: Our case showed, that despite the rarity of the tumor, it is important to consider pheochromocytoma in any patient with unexplained cardiogenic shock or left ventricular failure, multi-organ failure, hypertensive crisis or unexplained lactate acidosis, especially, if also febrile.