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New Perspectives on Pheochromocytoma and Paraganglioma: Toward a Molecular Classification
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Endocrine Tumor Biology. Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland. (Endokrin Tumörbiologi)
Aix Marseille Université, La Timone University Hospital, Department of Nuclear Medicine, European Center for Research in Medical Imaging, Marseille.
Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland.
2017 (English)In: Endocrine reviews, ISSN 0163-769X, E-ISSN 1945-7189, Vol. 38, no 6, p. 489-515Article, review/survey (Refereed) Published
Abstract [en]

A molecular biology-based taxonomy has been proposed for pheochromocytoma and paraganglioma (PPGL). Data from the Cancer Genome Atlas revealed clinically relevant prognostic and predictive biomarkers and stratified PPGLs into three main clusters. Each subgroup has a distinct molecular-biochemical-imaging signature. Concurrently, new methods for biochemical analysis, functional imaging, and medical therapies have also become available. The research community now strives to match the cluster biomarkers with the best intervention. The concept of precision medicine has been long awaited and holds great promise for improved care. Here, we review the current and future PPGL classifications, with a focus on hereditary syndromes. We discuss the current strengths and shortcomings of precision medicine and suggest a condensed manual for diagnosis and treatment of both adult and pediatric patients with PPGL. Finally, we consider the future direction of this field, with a particular focus on how advanced molecular characterization of PPGL can improve a patient's outcome, including cures and, ultimately, disease prevention.

Place, publisher, year, edition, pages
2017. Vol. 38, no 6, p. 489-515
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Endocrinology and Diabetes
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URN: urn:nbn:se:uu:diva-342325DOI: 10.1210/er.2017-00062ISI: 000419059200001PubMedID: 28938417OAI: oai:DiVA.org:uu-342325DiVA, id: diva2:1184062
Available from: 2018-02-20 Created: 2018-02-20 Last updated: 2018-03-26Bibliographically approved

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