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Metastatic Pheochromocytomas and Abdominal Paragangliomas
CONTEXT: Pheochromocytomas and paragangliomas (PPGLs) are believed to harbor malignant potential; about 10% to 15% of pheochromocytomas and up to 50% of abdominal paragangliomas will exhibit metastatic behavior. EVIDENCE ACQUISITION: Extensive searches in the PubMed database with various combination...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8063253/ https://www.ncbi.nlm.nih.gov/pubmed/33462603 http://dx.doi.org/10.1210/clinem/dgaa982 |
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author | Granberg, Dan Juhlin, Carl Christofer Falhammar, Henrik |
author_facet | Granberg, Dan Juhlin, Carl Christofer Falhammar, Henrik |
author_sort | Granberg, Dan |
collection | PubMed |
description | CONTEXT: Pheochromocytomas and paragangliomas (PPGLs) are believed to harbor malignant potential; about 10% to 15% of pheochromocytomas and up to 50% of abdominal paragangliomas will exhibit metastatic behavior. EVIDENCE ACQUISITION: Extensive searches in the PubMed database with various combinations of the key words pheochromocytoma, paraganglioma, metastatic, malignant, diagnosis, pathology, genetic, and treatment were the basis for the present review. DATA SYNTHESIS: To pinpoint metastatic potential in PPGLs is difficult, but nevertheless crucial for the individual patient to receive tailor-made follow-up and adjuvant treatment following primary surgery. A combination of histological workup and molecular predictive markers can possibly aid the clinicians in this aspect. Most patients with PPGLs have localized disease and may be cured by surgery. Plasma metanephrines are the main biochemical tests. Genetic testing is important, both for counseling and prognostic estimation. Apart from computed tomography and magnetic resonance imaging, molecular imaging using (68)Ga-DOTATOC/DOTATATE should be performed. (123)I-MIBG scintigraphy may be performed to determine whether (131)I-MIBG therapy is a possible option. As first-line treatment in patients with metastatic disease, (177)Lu-DOTATATE or (131)I-MIBG is recommended, depending on which shows best expression. In patients with very low proliferative activity, watch-and-wait or primary treatment with long-acting somatostatin analogues may be considered. As second-line treatment, or first-line in patients with high proliferative rate, chemotherapy with temozolomide or cyclophosphamide + vincristine + dacarbazine is the therapy of choice. Other therapies, including sunitinib, cabozantinib, everolimus, and PD-1/PDL-1 inhibitors, have shown modest effect. CONCLUSIONS: Metastatic PPGLs need individualized management and should always be discussed in specialized and interdisciplinary tumor boards. Further studies and newer treatment modalities are urgently needed. |
format | Online Article Text |
id | pubmed-8063253 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-80632532021-04-28 Metastatic Pheochromocytomas and Abdominal Paragangliomas Granberg, Dan Juhlin, Carl Christofer Falhammar, Henrik J Clin Endocrinol Metab Mini-Reviews CONTEXT: Pheochromocytomas and paragangliomas (PPGLs) are believed to harbor malignant potential; about 10% to 15% of pheochromocytomas and up to 50% of abdominal paragangliomas will exhibit metastatic behavior. EVIDENCE ACQUISITION: Extensive searches in the PubMed database with various combinations of the key words pheochromocytoma, paraganglioma, metastatic, malignant, diagnosis, pathology, genetic, and treatment were the basis for the present review. DATA SYNTHESIS: To pinpoint metastatic potential in PPGLs is difficult, but nevertheless crucial for the individual patient to receive tailor-made follow-up and adjuvant treatment following primary surgery. A combination of histological workup and molecular predictive markers can possibly aid the clinicians in this aspect. Most patients with PPGLs have localized disease and may be cured by surgery. Plasma metanephrines are the main biochemical tests. Genetic testing is important, both for counseling and prognostic estimation. Apart from computed tomography and magnetic resonance imaging, molecular imaging using (68)Ga-DOTATOC/DOTATATE should be performed. (123)I-MIBG scintigraphy may be performed to determine whether (131)I-MIBG therapy is a possible option. As first-line treatment in patients with metastatic disease, (177)Lu-DOTATATE or (131)I-MIBG is recommended, depending on which shows best expression. In patients with very low proliferative activity, watch-and-wait or primary treatment with long-acting somatostatin analogues may be considered. As second-line treatment, or first-line in patients with high proliferative rate, chemotherapy with temozolomide or cyclophosphamide + vincristine + dacarbazine is the therapy of choice. Other therapies, including sunitinib, cabozantinib, everolimus, and PD-1/PDL-1 inhibitors, have shown modest effect. CONCLUSIONS: Metastatic PPGLs need individualized management and should always be discussed in specialized and interdisciplinary tumor boards. Further studies and newer treatment modalities are urgently needed. Oxford University Press 2021-01-19 /pmc/articles/PMC8063253/ /pubmed/33462603 http://dx.doi.org/10.1210/clinem/dgaa982 Text en © The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) ), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | Mini-Reviews Granberg, Dan Juhlin, Carl Christofer Falhammar, Henrik Metastatic Pheochromocytomas and Abdominal Paragangliomas |
title | Metastatic Pheochromocytomas and Abdominal Paragangliomas |
title_full | Metastatic Pheochromocytomas and Abdominal Paragangliomas |
title_fullStr | Metastatic Pheochromocytomas and Abdominal Paragangliomas |
title_full_unstemmed | Metastatic Pheochromocytomas and Abdominal Paragangliomas |
title_short | Metastatic Pheochromocytomas and Abdominal Paragangliomas |
title_sort | metastatic pheochromocytomas and abdominal paragangliomas |
topic | Mini-Reviews |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8063253/ https://www.ncbi.nlm.nih.gov/pubmed/33462603 http://dx.doi.org/10.1210/clinem/dgaa982 |
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