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Clinical and Translational Oncology
Published in: Clinical and Translational Oncology 10/2021

Open Access 01-10-2021 | Special Article

Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas

Authors: R. Garcia-Carbonero, F. Matute Teresa, E. Mercader-Cidoncha, M. Mitjavila-Casanovas, M. Robledo, I. Tena, C. Alvarez-Escola, M. Arístegui, M. R. Bella-Cueto, C. Ferrer-Albiach, F. A. Hanzu

Published in: Clinical and Translational Oncology | Issue 10/2021

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Abstract

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). We will review the following topics: epidemiology; anatomy, pathology and molecular pathways; clinical presentation; hereditary predisposition syndromes and genetic counseling and testing; diagnostic procedures, including biochemical testing and imaging studies; treatment including catecholamine blockade, surgery, radiotherapy and radiometabolic therapy, systemic therapy, local ablative therapy and supportive care. Finally, we will provide follow-up recommendations.
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Metadata
Title
Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas
Authors
R. Garcia-Carbonero
F. Matute Teresa
E. Mercader-Cidoncha
M. Mitjavila-Casanovas
M. Robledo
I. Tena
C. Alvarez-Escola
M. Arístegui
M. R. Bella-Cueto
C. Ferrer-Albiach
F. A. Hanzu
Publication date
01-10-2021
Publisher
Springer International Publishing
Published in
Clinical and Translational Oncology / Issue 10/2021
Print ISSN: 1699-048X
Electronic ISSN: 1699-3055
DOI
https://doi.org/10.1007/s12094-021-02622-9

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