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Review
. 2014 Jan-Feb;38(1):7-41.
doi: 10.1016/j.currproblcancer.2014.01.001. Epub 2014 Jan 15.

Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment

Victoria L Martucci, Karel Pacak
Review

Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment

Victoria L Martucci et al. Curr Probl Cancer. 2014 Jan-Feb.
No abstract available

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Figures

Figure 1
Figure 1
Recommended genetic testing algorithm for patients with PHEO/PGL. (Adapted from Karasek et al. 2013) *If both normetanephrine and methoxytyramine are elevated, follow the algorithm for methoxytyramine. If both normetanephrine and metanephrine are elevated, follow the algorithm for metanephrine. **In patients with elevated normetanephrine with clinical features that do not clearly indicate which gene to test, perform immunohistochemistry for SDHB and SDHA. +If tumor is adrenal, TMEM127 testing may be considered. Abbreviations: DA: dopamine; HIF2A: hypoxia-inducible factor 2-alpha; HNP: head and neck PGL; h/o: history of; MAX: myc-associated factor X; MEN2: multiple endocrine neoplasia type 2; MTY: methoxytyramine; NF1: neurofibromatosis type 1; RET: rearranged during transfection; SDHA: succinate dehydrogenase subunit A; SDHB: succinate dehydrogenase subunit B; SDHC: succinate dehydrogenase subunit C; SDHD: succinate dehydrogenase subunit D; TMEM127: transmembrane protein 127; VHL: von Hippel-Lindau
Figure 2
Figure 2
Recommended functional imaging algorithm for patients with PHEO/PGL. Abbreviations: FDA: 18F-fluorodopamine; FDG: 18F-fluorodeoxyglucose; FDOPA: 18F-fluorodopa; 68Ga-DOTA: 68Ga-DOTA-peptides; 123I-MIBG: 123I-metaiodobenzylguanidine; PET: positron emission tomography;; SDHB: succinate dehydrogenase subunit B; SDHx: succinate dehydrogenase mutation
See this image and copyright information in PMC

References

    1. Chen H, Sippel RS, O'Dorisio MS, et al. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas. 2010;39:775–783. - PMC - PubMed
    1. McNeil AR, Blok BH, Koelmeyer TD, et al. Phaeochromocytomas discovered during coronial autopsies in Sydney, Melbourne and Auckland. Aust N Z J Med. 2000;30:648–652. - PubMed
    1. Anderson GH, Blakeman N, Streeten DH. The effect of age on prevalence of secondary forms of hypertension in 4429 consecutively referred patients. J Hypertens. 1994;12:609–615. - PubMed
    1. Omura M, Saito J, Yamaguchi K, et al. Prospective study on the prevalence of secondary hypertension among hypertensive patients visiting a general outpatient clinic in Japan. Hypertens Res. 2004;27:193–202. - PubMed
    1. Lenders JWM, Eisenhofer G, Mannelli M, et al. Phaeochromocytoma. Lancet. 2005;366:665–675. - PubMed

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