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...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2022-10-01
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| Series: | Frontiers in Endocrinology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fendo.2022.1021420/full |
| _version_ | 1798031265992015872 |
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| author | Georgiana Constantinescu Georgiana Constantinescu Georgiana Constantinescu Cristina Preda Victor Constantinescu Timo Siepmann Timo Siepmann Stefan R. Bornstein Stefan R. Bornstein Stefan R. Bornstein Jacques W. M. Lenders Jacques W. M. Lenders Graeme Eisenhofer Graeme Eisenhofer Christina Pamporaki |
| author_facet | Georgiana Constantinescu Georgiana Constantinescu Georgiana Constantinescu Cristina Preda Victor Constantinescu Timo Siepmann Timo Siepmann Stefan R. Bornstein Stefan R. Bornstein Stefan R. Bornstein Jacques W. M. Lenders Jacques W. M. Lenders Graeme Eisenhofer Graeme Eisenhofer Christina Pamporaki |
| author_sort | Georgiana Constantinescu |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-04-11T19:54:44Z |
| format | Article |
| id | doaj.art-585bfb077a064157b386f9aa3efa1218 |
| institution | Directory Open Access Journal |
| issn | 1664-2392 |
| language | English |
| last_indexed | 2024-04-11T19:54:44Z |
| publishDate | 2022-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Endocrinology |
| spelling | doaj.art-585bfb077a064157b386f9aa3efa12182022-12-22T04:06:12ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922022-10-011310.3389/fendo.2022.10214201021420Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminologyGeorgiana Constantinescu0Georgiana Constantinescu1Georgiana Constantinescu2Cristina Preda3Victor Constantinescu4Timo Siepmann5Timo Siepmann6Stefan R. Bornstein7Stefan R. Bornstein8Stefan R. Bornstein9Jacques W. M. Lenders10Jacques W. M. Lenders11Graeme Eisenhofer12Graeme Eisenhofer13Christina Pamporaki14Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyDepartment of Endocrinology, Grigore T. Popa University, Iasi, RomaniaDepartment of Health Care Sciences, Center for Clinical Research and Management Education, Dresden Inter-national University, Dresden, GermanyDepartment of Endocrinology, Grigore T. Popa University, Iasi, RomaniaCenter of Clinical Neuroscience, University Clinic Carl-Gustav Carus, Dresden University of Technology, Dresden, GermanyDepartment of Health Care Sciences, Center for Clinical Research and Management Education, Dresden Inter-national University, Dresden, GermanyDepartment of Neurology, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyDepartment of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyDepartment of Health Care Sciences, Center for Clinical Research and Management Education, Dresden International University, Dresden, GermanyDivision of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King's College London, London, United KingdomDepartment of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyDepartment of Internal Medicine, Radboud University Medical Centre, Nijmegen, NetherlandsDepartment of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyInstitute of Clinical Chemistry and Laboratory Medicine, University of Dresden, Dresden, GermanyDepartment of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyPheochromocytomas 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.https://www.frontiersin.org/articles/10.3389/fendo.2022.1021420/fullpheochromocytomaparagangliomassilentclinically silentbiochemically negative |
| spellingShingle | Georgiana Constantinescu Georgiana Constantinescu Georgiana Constantinescu Cristina Preda Victor Constantinescu Timo Siepmann Timo Siepmann Stefan R. Bornstein Stefan R. Bornstein Stefan R. Bornstein Jacques W. M. Lenders Jacques W. M. Lenders Graeme Eisenhofer Graeme Eisenhofer Christina Pamporaki Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology Frontiers in Endocrinology pheochromocytoma paragangliomas silent clinically silent biochemically negative |
| 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 | pheochromocytoma paragangliomas silent clinically silent biochemically negative |
| url | https://www.frontiersin.org/articles/10.3389/fendo.2022.1021420/full |
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