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, 38 (1), 7-41

Pheochromocytoma and Paraganglioma: Diagnosis, Genetics, Management, and Treatment

Review

Pheochromocytoma and Paraganglioma: Diagnosis, Genetics, Management, and Treatment

Victoria L Martucci et al. Curr Probl Cancer.

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

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