Next Article in Journal
Whole Tumor Histogram Analysis Using DW MRI in Primary Central Nervous System Lymphoma Correlates with Tumor Biomarkers and Outcome
Next Article in Special Issue
Orthopedia Homeobox (OTP) in Pulmonary Neuroendocrine Tumors: The Diagnostic Value and Possible Molecular Interactions
Previous Article in Journal
Clinical Practice Use of Liquid Biopsy to Identify RAS/BRAF Mutations in Patients with Metastatic Colorectal Cancer (mCRC): A Single Institution Experience
Previous Article in Special Issue
Management of Small Bowel Neuroendocrine Tumors
Open AccessReview

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

1
Department of Medicine IV, University Hospital, LMU Munich, Ziemssenstraße 1, 80336 München, Germany
2
Department of Radiopharmaceutical and Chemical Biology, Institute of Radiopharmaceutical Cancer Research, Helmholtz-Zentrum Dresden-Rossendorf, Bautzner Landstrasse 400, 01328 Dresden, Germany
3
Department of Chemistry and Food Chemistry, School of Science, Technische Universität Dresden, Mommsenstrasse 9, 01062 Dresden, Germany
4
Department of Medicine III, University Hospital Carl Gustav Carus Dresden, Fetscherstraße 74, 01307 Dresden, Germany
5
Institute for Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus at Technische Universität Dresden, 01307 Dresden, Germany
6
Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford Ox3 7LJ, UK
7
Department of Gastroenterology, Royal Free Hospital ENETS Centre of Excellence, London NW3 2QG, UK
8
Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20814, USA
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
Cancers 2019, 11(10), 1505; https://doi.org/10.3390/cancers11101505
Received: 21 August 2019 / Revised: 18 September 2019 / Accepted: 24 September 2019 / Published: 8 October 2019
(This article belongs to the Special Issue Management of Neuroendocrine Neoplasms)
Pheochromocytomas and paragangliomas (PCC/PGLs) are rare, mostly catecholamine-producing neuroendocrine tumors of the adrenal gland (PCCs) or the extra-adrenal paraganglia (PGL). They can be separated into three different molecular clusters depending on their underlying gene mutations in any of the at least 20 known susceptibility genes: The pseudohypoxia-associated cluster 1, the kinase signaling-associated cluster 2, and the Wnt signaling-associated cluster 3. In addition to tumor size, location (adrenal vs. extra-adrenal), multiplicity, age of first diagnosis, and presence of metastatic disease (including tumor burden), other decisive factors for best clinical management of PCC/PGL include the underlying germline mutation. The above factors can impact the choice of different biomarkers and imaging modalities for PCC/PGL diagnosis, as well as screening for other neoplasms, staging, follow-up, and therapy options. This review provides a guide for practicing clinicians summarizing current management of PCC/PGL according to tumor size, location, age of first diagnosis, presence of metastases, and especially underlying mutations in the era of precision medicine. View Full-Text
Keywords: pheochromocytoma; paraganglioma; guideline; genetics; diagnosis; biomarkers; imaging; follow-up; therapy; precision medicine pheochromocytoma; paraganglioma; guideline; genetics; diagnosis; biomarkers; imaging; follow-up; therapy; precision medicine
Show Figures

Figure 1

MDPI and ACS Style

Nölting, S.; Ullrich, M.; Pietzsch, J.; Ziegler, C.G.; Eisenhofer, G.; Grossman, A.; Pacak, K. Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine. Cancers 2019, 11, 1505.

Show more citation formats Show less citations formats
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Back to TopTop