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Targeted lipidomics between hPheo1 and SDHB KD cells reveal changes in bioactive lipids and PKC with polyamine pathway inhibition.

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The Biochemical Spectrum of Pheochromocytoma.

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Pheochromocytomas and Paragangliomas.

Sergei G Tevosian1, Hans K Ghayee2

  • 1Department of Physiological Sciences, College of Veterinary Medicine, University of Florida, 1600 Southwest Archer Road, Suite H-2, Gainesville, FL 32608, USA.

Endocrinology and Metabolism Clinics of North America
|October 28, 2019
PubMed
Summary

Pheochromocytomas and paragangliomas are rare tumors causing excess catecholamines, leading to hypertension. This article reviews current clinical approaches for diagnosing and managing these rare neuroendocrine tumors.

Keywords:
Extra-adrenal pheochromocytomaHypertensionNeuroendocrine tumorsParagangliomasPheochromocytomas

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Area of Science:

  • Endocrinology
  • Oncology
  • Nephrology

Background:

  • Pheochromocytomas and paragangliomas are rare neuroendocrine tumors originating from autonomic neural ganglia.
  • Excess catecholamine secretion is the hallmark of these tumors, frequently causing severe hypertension.
  • While rare, these tumors are a significant secondary cause of hypertension, necessitating clinical suspicion.

Purpose of the Study:

  • To summarize current clinical diagnostic and management strategies for pheochromocytoma and paraganglioma.
  • To highlight the critical role of these tumors as a secondary cause of hypertension.
  • To provide an overview of the clinical presentation and approach to patients with suspected pheochromocytoma/paraganglioma.

Main Methods:

  • Review of current clinical guidelines and literature on pheochromocytoma and paraganglioma.
  • Summary of diagnostic workup, including biochemical testing and imaging modalities.
  • Outline of therapeutic options, encompassing medical and surgical management.

Main Results:

  • Hypertension is the most common and critical symptom, with a prevalence of 0.1%–0.6% in hypertensive patients.
  • Clinical suspicion is often raised when pheochromocytoma/paraganglioma is considered a secondary cause of hypertension.
  • Effective diagnostic and management strategies are crucial for patient outcomes.

Conclusions:

  • Early recognition and diagnosis of pheochromocytoma/paraganglioma are vital, especially in hypertensive patients.
  • A multidisciplinary approach involving endocrinologists, surgeons, and radiologists is often required.
  • Current clinical strategies focus on accurate diagnosis and tailored management to control catecholamine excess and hypertension.