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What's New in Endocrinology?

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Front. Endocrinol. | doi: 10.3389/fendo.2018.00515

Update of Pheochromocytoma Syndromes: Genetics, Biochemical Evaluation, and Imaging

  • 1Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health (NIH), United States
  • 2National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health (NIH), United States
  • 3Consorcio Hospitalario Provincial de Castellón, Spain

Pheochromocytomas (PCCs) are tumors of the chromaffin cells that arises within the adrenal medulla, whereas, paragangliomas (PGLs) are neural crest-derived neuroendocrine neoplasms arising from the extrarenal paraganglia. Pheochromocytomas and paragangliomas syndromes (PPGLs) are rapidly evolving field in endocrinology and oncology. Discoveries over the last decade and especially over the last few years has significantly improved our understanding of PPGLs. Recent advances included the findings of newly related syndromes in PPGL and its associated susceptibility genes. Besides, new radiological functional studies and targeted radionuclide therapy have improved the diagnostic accuracy and provided new tools for treatment. In this review article, we will present recent advances in this field and recommend new biochemical classification that better reflect our new understanding of the disease.

Keywords: Pheochromocytoma, Paraganglioma, TCGA, MDH2, PHD1, IRP1, ATRX, Catecholamines, Metanephrines, DOTATATE, PRRT, DOTA-JR11, endocrine tumors

Received: 24 Apr 2018; Accepted: 16 Aug 2018.

Edited by:

Derek LeRoith, Icahn School of Medicine at Mount Sinai, United States

Reviewed by:

AGNESE BARNABEI, Istituto Nazionale del Cancro Regina Elena, Italy
Luiz E. Wildemberg, Instituto Estadual do Cérebro Paulo Niemeyer, Brazil  

Copyright: © 2018 Suarez, Alrezk, Tena and Pacak. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Prof. Karel Pacak, National Institutes of Health (NIH), Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, United States,