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Hypersecretory Paraganglioma Presenting as Acute Aortic Dissection.
Christian J Lorenzo1, Qassem K Abdelal1, Jorge I Conte1
1Department of Medicine, Sarasota Memorial Hospital, Florida State University College of Medicine, Sarasota, Florida.
JACC. Case Reports
|July 28, 2021
Summary
Catecholamine-secreting tumors can cause severe hypertension and acute aortic dissection. This case highlights successful multidisciplinary management of an extra-adrenal paraganglioma presenting as aortic dissection.
Area of Science:
- Cardiovascular Medicine
- Endocrinology
- Oncology
Background:
- Severe hypertension, often episodic, can arise from catecholamine-secreting tumors like pheochromocytomas and paragangliomas.
- These hypertensive crises pose a significant risk for acute cardiovascular events, including aortic dissection.
- Extra-adrenal paragangliomas are rare neuroendocrine tumors that can secrete vasoactive substances.
Observation:
- A patient presented with acute aortic dissection, a life-threatening condition.
- Diagnostic workup revealed an underlying, previously undiagnosed, extra-adrenal paraganglioma.
- The tumor was identified as the source of catecholamine excess leading to severe hypertension.
Findings:
- The patient experienced abrupt, severe hypertension due to the functional paraganglioma.
- This hypertensive emergency precipitated the acute aortic dissection.
- Successful surgical resection of the paraganglioma and management of the aortic dissection were achieved.
Implications:
- This case underscores the critical importance of considering catecholamine-secreting tumors in patients presenting with unexplained severe hypertension and aortic emergencies.
- Multidisciplinary management involving endocrinology, cardiology, and cardiothoracic surgery is crucial for optimal outcomes.
- Early diagnosis and treatment of functional paragangliomas can prevent catastrophic cardiovascular complications such as aortic dissection.
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