Adrenal Incidentaloma with Cardiogenic Shock; How to Manage a Possible Phaechromocytoma?

Presentation Number: SAT 397
Date of Presentation: April 1st, 2017

Sushuma Kalidindi*1, Diane Barker2, Arun M A Vijay1, Feaz Babwah1, Fraz Umar2, Cherian George1, John Oxtoby1, Julian Waldron1, Anthony A Fryer3 and Fahmy WF Hanna1
1University Hospitals of North Midlands NHS Trust, United Kingdom, 2University Hospitals of North Midlands NHS Trust, 3University Hospitals of North Midlands NHS Trust, Stoke-on-Trent, United Kingdom

Abstract

Introduction:
Catecholamine-secreting tumours are rare neoplasms. Rarely pheochromocytoma is associated with cardiomyopathy attributed to catecholamine excess; this is similar to stress induced cardiomyopathy1. We present the case of a patient who presented with acute heart failure and was noted to have a 4.6cm adrenal lesion.

Clinical case:
A 51-year old male presented on a weekend with chest pain. He had an 18 month history of headaches and flushing episodes. He is on treatment for hypertension and had no other significant past medical or family history. On examination he was in overt heart failure.

Results:
His troponin was 23,488 ng/L (0-39ng/L). Transthoracic echocardiogram showed a global left ventricular systolic dysfunction, ejection fraction of 10%. All walls were almost akinetic. Cardiac MRI excluded a myocardial infarction. CT aortogram excluded dissection, however an incidental indeterminate, lipid poor right adrenal lesion was found.
The adrenal lesion along with the presentation gave the clinical appearance of a pheochromocytoma. Plasma and urine metanephrines were requested, although they can take up to a week to be reported. Urine metadrenaline which were reported at a later stage were markedly elevated at 89,408 nmol/24h (0-2000 nmol/24h).

Management:
Acute heart failure was treated with diuretics. His case was discussed with the cardiac transplant centre in case he failed to respond to medical treatment. As the patient was in extremis, we had to proceed with coronary angiography without metanephrines results. In this setting, as a life saving measure, we started an alpha blocker and proceeded cautiously, expecting worsening of crisis and considered other agents (eg-nitroprusside infusion if needed). The coronary angiogram was uneventful and revealed normal coronary arteries.

Discussion:
This case highlights:
1. The necessity of multidisciplinary approach when dealing with possible pheochromocytoma. This case required the direct interaction of the consultant cardiologist, endocrinologist, radiologist and clinical biochemist to discuss the findings and plan the necessary investigations.
2. The need to carefully consider the pros and cons of an intensive intervention (coronary angiogram) in a patient with possible pheochromocytoma. This can be a lifesaving measure, but needed to be done before the results of the urinary/plasma metanephrines, especially as the patient presented over a weekend.
3. Urinary metanephrines require a full 24 hour accurate collection with restrictions. The analysis is only done once per week in our centre. Plasma metanephrines are often sent to a reference lab and therefore take more than 4 weeks to come back. This adds pressure on the handling of acute presentations like our case. We needed to ensure multi-disciplinary interaction and full engagement with patient, family to appreciate the uncertainties and impact on decision making.

 

Nothing to Disclose: SK, DB, AMAV, FB, FU, CG, JO, JW, AAF, FWH