Endocrine Abstracts

Introduction: Pheochromocytomas and paragangliomas (PPGLs) are rare tumors of the chromaffin tissue characterised by catecholamine excess. Cardiovascular complications, such as hypertensive crisis and catecholamine-induced cardiomyopathy, are known to be the most frequent causes of life-threatening events in PPGLs patients.

Design: We analysed records of patients diagnosed with PPGLs in one referral centre from Romania between 1976 and 2021 (n= 106) in order to compare demographics, symptoms, preoperative catecholamine levels, tumor diameter and outcome in patients with life-threatening events vs. patients without complications (control group).

Results: 9 patients (8.4%), 8 F, 1 M presented life-threatening events before the diagnosis or during surgery for PPGL: catecholamine-induced Tako-Tsubo cardiomyopathy (n=3); inverted Tako-Tsubo cardiomyopathy and pulmonary edema (n=1); acute coronary syndrome and pulmonary edema (n=2); pulmonary edema (n=1); cardiac arrest and pulmonary edema (n=1); pulmonary thromboembolism and feto-placental apoplexy (n=1). Seven patients had the life-threatening event before the diagnosis of PPGL and two of them had the complications during surgery for PPGL. Compared to pheochromocytoma patients without life-threatening events (n= 97), patients with severe complications had a nonsignificantly higher mean tumor diameter (53 vs. 48 cm, P=0.3), similar levels of catecholamine (median 5-6 fold ULN; P=0.5), similar age at diagnosis (50 vs. 47 years; P=0.51). The maximum arterial blood pressure before surgery was non-significantly higher in patients with complication (231 vs. 207 mmHg; P=0.1). All patients with complications survived. Patients with Tako-Tsubo cardiomiopathy had normal cardiac function few days after the event. One patient with acute coronary syndrome and pulmonary edema but a background of other cardiac comorbidities had the ejection fraction of 40% after this event. The woman with feto-placental apoplexy had a negative outcome: her baby died in uterus and she underwent hysterectomy at 34 years old.

Conclusion: Although pheochromocytomas are rare tumors, they may induce a life-threatening complications (diagnosed in 8.4% of cases in our series). There was no specific clinical, hormonal or imaging feature that could predict a life-threatening event in patients with PPGLs.