Cargando…

Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with heterogeneous clinical presentations and potential lethal outcomes. The diagnosis is based on clinical suspicion, biochemical testing, imaging and histopathological confirmation. Increasingly widespread use of imaging s...

Descripción completa

Detalles Bibliográficos
Autores principales: Constantinescu, Georgiana, Preda, Cristina, Constantinescu, Victor, Siepmann, Timo, Bornstein, Stefan R., Lenders, Jacques W. M., Eisenhofer, Graeme, Pamporaki, Christina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9618947/
https://www.ncbi.nlm.nih.gov/pubmed/36325453
http://dx.doi.org/10.3389/fendo.2022.1021420
_version_ 1784821165889421312
author Constantinescu, Georgiana
Preda, Cristina
Constantinescu, Victor
Siepmann, Timo
Bornstein, Stefan R.
Lenders, Jacques W. M.
Eisenhofer, Graeme
Pamporaki, Christina
author_facet Constantinescu, Georgiana
Preda, Cristina
Constantinescu, Victor
Siepmann, Timo
Bornstein, Stefan R.
Lenders, Jacques W. M.
Eisenhofer, Graeme
Pamporaki, Christina
author_sort Constantinescu, Georgiana
collection PubMed
description Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with heterogeneous clinical presentations and potential lethal outcomes. The diagnosis is based on clinical suspicion, biochemical testing, imaging and histopathological confirmation. Increasingly widespread use of imaging studies and surveillance of patients at risk of PPGL due to a hereditary background or a previous tumor is leading to the diagnosis of these tumors at an early stage. This has resulted in an increasing use of the term “silent” PPGL. This term and other variants are now commonly found in the literature without any clear or unified definition. Among the various terms, “clinically silent” is often used to describe the lack of signs and symptoms associated with catecholamine excess. Confusion arises when these and other terms are used to define the tumors according to their ability to synthesize and/or release catecholamines in relation to biochemical test results. In such cases the term “silent” and other variants are often inappropriately and misleadingly used. In the present analysis we provide an overview of the literature and propose standardized terminology in an attempt at harmonization to facilitate scientific communication.
format Online
Article
Text
id pubmed-9618947
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Frontiers Media S.A.
record_format MEDLINE/PubMed
spelling pubmed-96189472022-11-01 Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology Constantinescu, Georgiana Preda, Cristina Constantinescu, Victor Siepmann, Timo Bornstein, Stefan R. Lenders, Jacques W. M. Eisenhofer, Graeme Pamporaki, Christina Front Endocrinol (Lausanne) Endocrinology Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with heterogeneous clinical presentations and potential lethal outcomes. The diagnosis is based on clinical suspicion, biochemical testing, imaging and histopathological confirmation. Increasingly widespread use of imaging studies and surveillance of patients at risk of PPGL due to a hereditary background or a previous tumor is leading to the diagnosis of these tumors at an early stage. This has resulted in an increasing use of the term “silent” PPGL. This term and other variants are now commonly found in the literature without any clear or unified definition. Among the various terms, “clinically silent” is often used to describe the lack of signs and symptoms associated with catecholamine excess. Confusion arises when these and other terms are used to define the tumors according to their ability to synthesize and/or release catecholamines in relation to biochemical test results. In such cases the term “silent” and other variants are often inappropriately and misleadingly used. In the present analysis we provide an overview of the literature and propose standardized terminology in an attempt at harmonization to facilitate scientific communication. Frontiers Media S.A. 2022-10-17 /pmc/articles/PMC9618947/ /pubmed/36325453 http://dx.doi.org/10.3389/fendo.2022.1021420 Text en Copyright © 2022 Constantinescu, Preda, Constantinescu, Siepmann, Bornstein, Lenders, Eisenhofer and Pamporaki https://creativecommons.org/licenses/by/4.0/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.
spellingShingle Endocrinology
Constantinescu, Georgiana
Preda, Cristina
Constantinescu, Victor
Siepmann, Timo
Bornstein, Stefan R.
Lenders, Jacques W. M.
Eisenhofer, Graeme
Pamporaki, Christina
Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology
title Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology
title_full Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology
title_fullStr Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology
title_full_unstemmed Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology
title_short Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology
title_sort silent pheochromocytoma and paraganglioma: systematic review and proposed definitions for standardized terminology
topic Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9618947/
https://www.ncbi.nlm.nih.gov/pubmed/36325453
http://dx.doi.org/10.3389/fendo.2022.1021420
work_keys_str_mv AT constantinescugeorgiana silentpheochromocytomaandparagangliomasystematicreviewandproposeddefinitionsforstandardizedterminology
AT predacristina silentpheochromocytomaandparagangliomasystematicreviewandproposeddefinitionsforstandardizedterminology
AT constantinescuvictor silentpheochromocytomaandparagangliomasystematicreviewandproposeddefinitionsforstandardizedterminology
AT siepmanntimo silentpheochromocytomaandparagangliomasystematicreviewandproposeddefinitionsforstandardizedterminology
AT bornsteinstefanr silentpheochromocytomaandparagangliomasystematicreviewandproposeddefinitionsforstandardizedterminology
AT lendersjacqueswm silentpheochromocytomaandparagangliomasystematicreviewandproposeddefinitionsforstandardizedterminology
AT eisenhofergraeme silentpheochromocytomaandparagangliomasystematicreviewandproposeddefinitionsforstandardizedterminology
AT pamporakichristina silentpheochromocytomaandparagangliomasystematicreviewandproposeddefinitionsforstandardizedterminology