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Endocrine Abstracts (2024) 100 WD4.4 | DOI: 10.1530/endoabs.100.WD4.4

1Hull University Teaching Hospitals NHS trust, Hull, United Kingdom; 2Dudley Group NHS Foundation Trust, Dudley, United Kingdom


A 63 year old male presented with sudden onset severe chest pain which clinically seemed cardiac in nature. A diagnosis of Non-ST Elevation MI was made and was managed conservatively. Later, after 6 months he again presented with chest pain. On this occasion, CT aorta and CT Thorax, Abdomen and Pelvis was done to rule out aortic aneurysmal rupture, and to look for post-infarct changes in the myocardium, cardiac MRI was done. Cardiac MRI and CTTAP both showed an incidental finding of left adrenal lesion measuring 4.8 cm highly suspicious of adrenal pheochromocytoma. On further exploration, he expressed having headache, sweating, palpitations and persistently raised blood pressure along with chest pain for past 6 months. Radiologists’ opinion was sought for further characterization of the mass. The lesion was suggested to have high vascularity due to rich capillary network, arterial phase enhancement and significant washout on PV phase. Investigations showed high levels of urinary and plasma metanephrines. Low-dose dexamethasone suppression test and overnight dexamethasone suppression test showed unsuppressed cortisol levels supporting possibility of co-secreting cortisol. Renin activity and aldosterone levels were normal. He was given adequate alpha blockade with increasing dose of doxazosin and was planned to have elective laparoscopic adrenalectomy. As an intraoperative complication, he developed grade 2 splenic rupture followed by a litre of blood loss due to which spleen was removed. Having sensitivity of 97% and specificity of 93%, the compelling evidence suggests using plasma free metanephrines as primary test of excessive catecholamines for diagnosis of Pheochromocytoma and Paragangliomas (PPGL). Computed Tomography is the first line investigation to confirm the location. PPGL should be ruled out in patients presenting with chest pain and persistently high blood pressure. Our patient did not have a family history of PPGL and the syndromes associated with it. However, one third of patients with PPGL have germline mutations and PPGL may often be a part of hereditary syndromes, so genetic testing is advisable for all the patients diagnosed with PPGL. The incidence of persistently low blood pressure after PPGL removal varies between 20-77% which is attributed to downregulation of alpha and beta receptors. Our patient developed persistent hypotension after the tumor removal which was managed with vasopressors in Intensive care.

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