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Phaeochromocytomas and functional paragangliomas: clinical management.
Best Pract Res Clin Endocrinol Metab. 2010 Dec; 24(6):933-41.BP

Abstract

Phaeochromocytomas (PH) and functional paragangliomas (FPGL) are neoplasms of adrenal (PH) or extra-adrenal (FPGL) chromaffin tissue that synthesize catecholamines. Catecholamines are converted into inactive metabolites, metanephrines, within the tumour and the diagnosis of PH/FPGL is therefore based on the quantification of plasma or urinary metanephrines. The tumour can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy. Patients are treated by tumour resection following alpha-blockade. PH and FPGL may be sporadic or part of several genetic diseases. Patients with PH/FPGL should be followed up indefinitely as the disease may recur, particularly if they have inherited or extra-adrenal tumours. About 10% of tumours are malignant either at initial surgery or during follow-up. Recurrences and malignancy are more frequent in cases with large or extra-adrenal tumours, and in SDHB mutation carriers. Treatments for progressive malignant PH/FPGL include tumour debulking, metabolic radiotherapy, chemotherapy, and possibly tyrosine kinase inhibitors.

Authors+Show Affiliations

Hypertension Unit, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris, France. pierre-francois.plouin@egp.aphp.frNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

21115162

Citation

Plouin, Pierre-François, et al. "Phaeochromocytomas and Functional Paragangliomas: Clinical Management." Best Practice & Research. Clinical Endocrinology & Metabolism, vol. 24, no. 6, 2010, pp. 933-41.
Plouin PF, Amar L, Lepoutre C. Phaeochromocytomas and functional paragangliomas: clinical management. Best Pract Res Clin Endocrinol Metab. 2010;24(6):933-41.
Plouin, P. F., Amar, L., & Lepoutre, C. (2010). Phaeochromocytomas and functional paragangliomas: clinical management. Best Practice & Research. Clinical Endocrinology & Metabolism, 24(6), 933-41. https://doi.org/10.1016/j.beem.2010.10.002
Plouin PF, Amar L, Lepoutre C. Phaeochromocytomas and Functional Paragangliomas: Clinical Management. Best Pract Res Clin Endocrinol Metab. 2010;24(6):933-41. PubMed PMID: 21115162.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Phaeochromocytomas and functional paragangliomas: clinical management. AU - Plouin,Pierre-François, AU - Amar,Laurence, AU - Lepoutre,Charlotte, PY - 2010/12/1/entrez PY - 2010/12/1/pubmed PY - 2011/3/22/medline SP - 933 EP - 41 JF - Best practice & research. Clinical endocrinology & metabolism JO - Best Pract Res Clin Endocrinol Metab VL - 24 IS - 6 N2 - Phaeochromocytomas (PH) and functional paragangliomas (FPGL) are neoplasms of adrenal (PH) or extra-adrenal (FPGL) chromaffin tissue that synthesize catecholamines. Catecholamines are converted into inactive metabolites, metanephrines, within the tumour and the diagnosis of PH/FPGL is therefore based on the quantification of plasma or urinary metanephrines. The tumour can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy. Patients are treated by tumour resection following alpha-blockade. PH and FPGL may be sporadic or part of several genetic diseases. Patients with PH/FPGL should be followed up indefinitely as the disease may recur, particularly if they have inherited or extra-adrenal tumours. About 10% of tumours are malignant either at initial surgery or during follow-up. Recurrences and malignancy are more frequent in cases with large or extra-adrenal tumours, and in SDHB mutation carriers. Treatments for progressive malignant PH/FPGL include tumour debulking, metabolic radiotherapy, chemotherapy, and possibly tyrosine kinase inhibitors. SN - 1878-1594 UR - https://www.unboundmedicine.com/medline/citation/21115162/Phaeochromocytomas_and_functional_paragangliomas:_clinical_management_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1521-690X(10)00129-6 DB - PRIME DP - Unbound Medicine ER -