# Preoperative Management of Pheochromocytoma and Paraganglioma

**Authors:** Fang Fang, Li Ding, Qing He, Ming Liu

PMC · DOI: 10.3389/fendo.2020.586795 · Frontiers in Endocrinology · 2020-09-29

## TL;DR

This paper reviews the preoperative management of pheochromocytoma and paraganglioma, focusing on preventing dangerous cardiovascular complications during surgery.

## Contribution

The paper summarizes recent studies comparing preoperative management with or without alpha-adrenergic-receptor antagonists.

## Key findings

- Proper preoperative management is crucial to prevent lethal cardiovascular complications during surgery.
- Use of alpha-adrenergic-receptor antagonists is a key part of preoperative preparation.
- Recent studies help clarify the effectiveness of various preoperative management strategies.

## Abstract

Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors, characterized by excessive release of catecholamines (CAs), and manifested as the classic triad of headaches, palpitations, profuse sweating, and a variety of other signs and symptoms. The diagnosis of PPGL requires both evidence of excessive release of CAs and anatomical localization of CA-secreting tumor. Surgery is the mainstay of treatment for all patients with PPGL unless contraindicated. However, without proper preparation, the release of excessive CAs, especially during surgery, can result in lethal cardiovascular complications. Herein, we briefly reviewed the pathogenesis of this disease, discussed the current approaches and evidence available for preoperative management, summarizing the results of the latest studies which compared the efficacies of preoperative management with or without α adrenergic-receptor antagonists, aiming to facilitate better understanding of the preoperative management of PPGL for the physicians.

## Linked entities

- **Diseases:** pheochromocytoma (MONDO:0004974), paraganglioma (MONDO:0000448)

## Full-text entities

- **Genes:** ADRB1 (adrenoceptor beta 1) [NCBI Gene 153] {aka ADRB1R, B1AR, BETA1AR, FNSS2, RHR}, AGT (angiotensinogen) [NCBI Gene 183] {aka ANHU, SERPINA8, hFLT1}, ADORA2A (adenosine A2a receptor) [NCBI Gene 135] {aka A2aR, ADORA2, RDC8}, GCG (glucagon) [NCBI Gene 2641] {aka GLP-1, GLP1, GLP2, GRPP}, MS4A1 (membrane spanning 4-domains A1) [NCBI Gene 931] {aka B1, Bp35, CD20, CVID5, FMC7, LEU-16}, AARS1 (alanyl-tRNA synthetase 1) [NCBI Gene 16] {aka AARS, CMT2N, DEE29, EIEE29, HDLS2, TTD8}, GPHA2 (glycoprotein hormone subunit alpha 2) [NCBI Gene 170589] {aka A2, GPA2, ZSIG51}, TH (tyrosine hydroxylase) [NCBI Gene 7054] {aka DYT14, DYT5b, TYH}, IGKV5-2 (immunoglobulin kappa variable 5-2) [NCBI Gene 28907] {aka B2, IGKV52}, BCL2A1 (BCL2 related protein A1) [NCBI Gene 597] {aka ACC-1, ACC-2, ACC1, ACC2, BCL2L5, BFL1}, NF1 (neurofibromin 1) [NCBI Gene 4763] {aka NFNS, VRNF, WSS}, AVP (arginine vasopressin) [NCBI Gene 551] {aka ADH, ARVP, AVP-NPII, AVRP, VP}, ADRB2 (adrenoceptor beta 2) [NCBI Gene 154] {aka ADRB2R, ADRBR, ARB2, B2AR, BAR, BETA2AR}
- **Diseases:** vomiting (MESH:D014839), renal dysfunction (MESH:D007674), depression (MESH:D003866), multiple endocrine neoplasia (MEN) type 2 (MESH:D018813), sweating (MESH:D013543), organ damage (MESH:D000092124), Palpitations (MESH:D006331), left ventricular hypertrophy (MESH:D017379), anxiety (MESH:D001007), cardiomyopathy (MESH:D009202), premature ventricular contraction (MESH:D018879), malignant hypertension (MESH:D006974), nasal congestion (MESH:D009668), mydriasis (MESH:D015878), atrioventricular block (MESH:D054537), allergy (MESH:D004342), catecholamine-secreting tumor (MESH:D009369), sick sinus syndrome (MESH:D012804), reflex tachycardia (MESH:D013610), Hyperglycemia (MESH:D006943), parkinsonism (MESH:D010302), hypotension (MESH:D007022), Edema (MESH:D004487), asthma (MESH:D001249), Bowel ischemia (MESH:D007511), coronary vasospasm (MESH:D003329), cardiovascular complications (MESH:D002318), cardiogenic shock (MESH:D012770), myocarditis (MESH:D009205), Angina (MESH:D000787), Hypertensive crisis (MESH:D006973), weight loss (MESH:D015431), CA (MESH:C536334), somnolence (MESH:D006970), hereditary syndromes (MESH:D009386), von Hippel-Lindau (VHL) syndrome (MESH:D006623), adrenal adenomas (MESH:D018246), cardiac arrest (MESH:D006323), glycosuria (MESH:D006029), headache (MESH:D006261), PPGL (MESH:D010673), retrograde ejaculation (MESH:D061686), heart or renal failure (MESH:D051437), Orthostatic hypotension (MESH:D007024), extrapyramidal signs (MESH:D001480), arrythmias (MESH:D001145), neuroendocrine tumor (MESH:D018358), adrenal incidentaloma (MESH:C538238), obesity (MESH:D009765), Blurry vision (MESH:D014786), pallor (MESH:D010167), renal artery dilatation (MESH:D012078), head and neck paragangliomas (MESH:D006258), bradycardia (MESH:D001919), hemodynamic instability (MESH:D043171), fatigue (MESH:D005221), Sympathetic paragangliomas (MESH:D010235), nausea (MESH:D009325), stiffness (MESH:C566112), shock (MESH:D012769)
- **Chemicals:** NMN (MESH:D009537), sodium nitroprusside (MESH:D009599), cocaine (MESH:D003042), steroids (MESH:D013256), Urapidil (MESH:C015568), CA synthesis inhibitors (-), E (MESH:D004540), histamine (MESH:D006632), labetalol (MESH:D007741), VMA (MESH:D014642), K+ (MESH:D011188), carvedilol (MESH:D000077261), DA (MESH:D004298), Nifedipine (MESH:D009543), Terazosin (MESH:C041226), Epinephrine (MESH:D004837), 123I-metaiodobenzylguanidine (MESH:D019797), phenylethylamine (MESH:D010627), tyrosine (MESH:D014443), alcohol (MESH:D000438), glucose (MESH:D005947), CA (MESH:D002395), MN (MESH:D008345), Metoprolol (MESH:D008790), DOPA (MESH:D004295), sodium (MESH:D012964), phentermine (MESH:D010645), Amlodipine (MESH:D017311), Magnesium sulfate (MESH:D008278), 18F-FDG (MESH:D019788), phentolamine (MESH:D010646), Doxazosin (MESH:D017292), normetanephrine (MESH:D009647), Phenoxybenzamine (MESH:D010643), metanephrine (MESH:D008676), Prazosin (MESH:D011224), Metyrosine (MESH:D019805), Nicardipine (MESH:D009529), amitriptyline (MESH:D000639), phendimetrazine (MESH:C100294), calcium (MESH:D002118), NE (MESH:D009638), Atenolol (MESH:D001262),  (MESH:D002121),  (MESH:D000317),  (MESH:D000319),  (MESH:D004791)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

58 references — full list in the complete paper: https://tomesphere.com/paper/PMC7551102/full.md

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Source: https://tomesphere.com/paper/PMC7551102