Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 May;27(5):1329-1337.
doi: 10.1245/s10434-020-08220-3. Epub 2020 Feb 28.

Update on Pheochromocytoma and Paraganglioma from the SSO Endocrine/Head and Neck Disease-Site Work Group. Part 1 of 2: Advances in Pathogenesis and Diagnosis of Pheochromocytoma and Paraganglioma

Dhaval Patel  1 , John E Phay  2 , Tina W F Yen  3 , Paxton V Dickson  4 , Tracy S Wang  3 , Roberto Garcia  5 , Anthony D Yang  6 , Carmen C Solórzano  7 , Lawrence T Kim  8
Affiliations
Review

Update on Pheochromocytoma and Paraganglioma from the SSO Endocrine/Head and Neck Disease-Site Work Group. Part 1 of 2: Advances in Pathogenesis and Diagnosis of Pheochromocytoma and Paraganglioma

Dhaval Patel et al. Ann Surg Oncol. 2020 May.

Abstract

This first part of a two-part review of pheochromocytoma and paragangliomas (PPGLs) addresses clinical presentation, diagnosis, management, treatment, and outcomes. In this first part, the epidemiology, prevalence, genetic etiology, clinical presentation, and biochemical and radiologic workup are discussed. In particular, recent advances in the genetics underlying PPGLs and the recommendation for genetic testing of all patients with PPGL are emphasized. Finally, the newer imaging methods for evaluating of PPGLs are discussed and highlighted.

PubMed Disclaimer

Figures

FIG. 1
FIG. 1
Chromaffin cells synthesize norepinephrine and epinephrine from phenylalanine, a tyrosine precursor. AAAD, L-aromatic amino acid decarboxylase; DBH, dopamine β-hydroxlase; L-DOPA, dihydroxyphenylalanine; PNMT, phenylethanolamine N-methyltransferase
FIG. 2
FIG. 2
A patient with a 3.34-cm right paraganglioma shown by a computed tomography (CT) axial slice and b CT coronal slice. The patient underwent a c 68-gallium dotatate scan, which showed high avidity
See this image and copyright information in PMC

References

    1. Lenders JW, Duh QY, Eisenhofer G, et al. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014;99:1915–42. - PubMed
    1. Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet. 2005;366:665–75. - PubMed
    1. Whalen RK, Althausen AF, Daniels GH. Extra-adrenal pheochromocytoma. J Urol. 1992;147:1–10. - PubMed
    1. Pacak K, Linehan WM, Eisenhofer G, Walther MM, Goldstein DS. Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma. Ann Intern Med. 2001;134:315–29. - PubMed
    1. Dailey L, Laplantine E, Priore R, Basilico C. A network of transcriptional and signaling events is activated by FGF to induce chondrocyte growth arrest and differentiation. J Cell Biol. 2003;161:1053–66. - PMC - PubMed

MeSH terms