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Pheochromocytoma/Paraganglioma: Update on Diagnosis and Management

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Book cover Adrenal Disorders

Part of the book series: Contemporary Endocrinology ((COE))

Abstract

After more than a century since they were described, pheochromocytomas and paragangliomas (PHEOs/PGLs) still pose great diagnostic and therapeutic challenges. Recent discoveries and progress in the understanding of molecular and metabolic changes associated with tumorigenesis allowed us to get closer to deciphering the secrets of these tumors. New knowledge allows us to develop novel diagnostic methods and identify specific treatment targets to improve the care of PHEO/PGL patients.

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Abbreviations

18F-DOPA:

18F-Dihydroxyphenylalanine

18F-FDA:

6-18F-Fluorodopamine

18F-FDG:

18F-Fluorodeoxyglucose

3PO:

3-(3-Pyridinyl)-1-(4-pyridinyl)-2-propen-1-on

68Ga-DOTATATE:

68Ga-DOTA(0)-Tyr(3)-octreotate

Acetyl-CoA:

Acetyl coenzyme A

ACLY:

ATP citrate lyase

AD:

Autosomal dominant

AM:

Morning

AMPK:

AMP-activated protein kinase

ATP:

Adenosine triphosphate

ATRX :

Alpha thalassemia/mental retardation syndrome X-linked

BCH:

2-Aminobicyclo(2,2,1)-heptane-2-carboxylic acid

BP:

Blood pressure

BPTES:

Bis-2-[5-phenylacetamido-1, 2, 4-thiadiazol-2-yl] ethyl sulfide

CHC:

α-Cyano-4-hydroxycinnamate

CT:

Computed tomography

CVD:

Cyclophosphamide, vincristine, dacarbazine

E:

Epinephrine

EBRT:

External beam radiation therapy

EGCG:

Epigallocatechin gallate

EGLN1/2 :

Egl-9 family hypoxia-inducible factor 1/2 (see also PHD2/1)

FH:

Fumarate hydratase

GDH1:

Glutamate dehydrogenase 1

GLUT:

Glucose transporter

GOT2:

Glutamate oxaloacetate transaminase 2

GPNA:

γ-L-Glutamyl-p-nitroanilide

GPT2:

Glutamate pyruvate transaminase 2

HIF:

Hypoxia-inducible factor

HIF2A :

Hypoxia-inducible factor 2 alpha

HK:

Hexokinase

HNPGL:

Head and neck paraganglioma

HPLC:

High-performance liquid chromatography

HR:

Heart rate

HRAS :

Harvey rat sarcoma viral oncogene homolog

IDH:

Isocitrate dehydrogenase

KIF1Bβ:

Kinesin family member 1B

LAT1:

L-type amino acid transporter 1

LDHA:

Lactate dehydrogenase A

MAX :

myc-associated factor X gene

MCT:

Monocarboxylase transporter

MDH2:

Malate dehydrogenase 2

MEN2A/2B:

Multiple endocrine neoplasia, type 2A/2B

MIBG:

Metaiodobenzylguanidine

MN:

Metanephrine

MRI:

Magnetic resonance imaging

mRNA:

Messenger RNA

MTY:

Methoxytyramine

NE:

Norepinephrine

NF1:

Neurofibromatosis type 1

NF1 :

Neurofibromin 1

NMN:

Normetanephrine

NS:

Nonsecreting

PDH:

Pyruvate dehydrogenase

PET:

Positron emission tomography

PFK:

Phosphofructokinase

PGC1α:

Peroxisome proliferator-activated receptor-γ coactivator 1α

PGL:

Paraganglioma

PHD1/2 :

HIF prolyl hydroxylase domain-containing protein 1/2 (see also EGLN2/1)

PHEO:

Pheochromocytoma

PI:

Paternal inheritance

PKM2:

Pyruvate kinase, isoenzyme 2

RECIST:

Response evaluation criteria in solid tumors

RET :

Rearranged during transfection proto-oncogene

RFA:

Radio-frequency ablation

ROS:

Reactive oxygen species

SDH:

Succinate dehydrogenase

SDHA, SDHB, SDHC, SDHD:

Succinate dehydrogenase subunits A, B, C, and D

SDHAF2 :

SDH assembly factor 2

TAPGL:

Thoracic and abdominal paraganglioma

TMEM127 :

Transmembrane protein 127

VEGF:

Vascular endothelial growth factor

VHL:

von Hippel-Lindau

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  1. Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, CRC, 1E-3140, 10 Center Drive, MSC-1109, Bethesda, MD, 20892-1109, USA

    Ivana Jochmanova M.D., M.Ed & Karel Pacak M.D., Ph.D., D.Sc

  2. 1st Department of Internal Medicine, Medical Faculty of P.J. Šafárik University in Košice, Trieda SNP 1, 04011, Košice, Slovakia

    Ivana Jochmanova M.D., M.Ed

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  1. Div of Endocrinology and Bone Diseases, Icahn School of Medicine at Mount Sinai, New York, New York, USA

    Alice C. Levine

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Jochmanova, I., Pacak, K. (2018). Pheochromocytoma/Paraganglioma: Update on Diagnosis and Management. In: Levine, A. (eds) Adrenal Disorders. Contemporary Endocrinology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-62470-9_12

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