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Pheochromocytomas and Hypertension
Joseph M Pappachan1, Nyo Nyo Tun2, Ganesan Arunagirinathan2
1Department of Endocrinology and Metabolism, University Hospitals of Morecambe Bay NHS Foundation Trust, Lancaster, LA1 4RP, UK. drpappachan@yahoo.co.in.
Current Hypertension Reports
|January 23, 2018
Summary
Pheochromocytomas and paragangliomas (PPGLs) are rare tumors causing hypertension. This review updates knowledge on their diagnosis, genetics, and treatment, emphasizing recent findings.
Area of Science:
- Endocrinology
- Oncology
- Genetics
Background:
- Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors originating from the adrenal medulla or autonomic ganglia.
- Catecholamine excess from PPGLs leads to hypertension, sweating, headaches, and palpitations, increasing cardiovascular risks.
- Genetic mutations are found in 35-40% of cases, with 10-15% of pheochromocytomas and 20-50% of paragangliomas being malignant.
Purpose of the Study:
- To provide an updated review of pheochromocytomas and paragangliomas (PPGLs).
- To cover the pathophysiology, genetic aspects, and diagnostic and therapeutic algorithms for PPGLs.
- To focus on scientific literature published within the past three years.
Main Methods:
- Literature review focusing on recent scientific publications (past 3 years).
- Analysis of diagnostic approaches including biochemical tests (plasma-free metanephrines, 24-h urine-fractionated metanephrines) and imaging (CT, MRI, 123I-MIBG, PET, SPECT).
- Review of current therapeutic strategies for both curative and palliative management.
Main Results:
- Biochemical diagnosis relies on highly sensitive and specific measurements of plasma or urinary metanephrines.
- Anatomical localization is typically achieved with CT or MRI, followed by functional imaging for prognostication.
- Management includes surgery, chemotherapy, radiotherapy, radionuclide therapy, and ablation procedures, with annual follow-up recommended.
Conclusions:
- PPGLs are significant causes of endocrine hypertension with unique neuroendocrine tumor characteristics.
- Updated diagnostic and therapeutic algorithms are crucial for managing these neoplasms.
- Genetic testing and comprehensive follow-up are essential for optimal patient outcomes.
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