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. 2023 Jan 13;56:101818. doi: 10.1016/j.eclinm.2022.101818

Table 12.

Guideline Recommendations for phaeochromocytoma and paraganglioma.

Phaeochromocytoma and paraganglioma
No Recommendations Strength
1 Routine biochemical screening for phaeochromocytoma and paraganglioma is not recommended in people with NF1 except for all women with NF1 who are contemplating pregnancy or are already pregnant. moderate
2 Biochemical testing for phaeochromocytoma and paraganglioma should be conducted in any person with NF1 who has raised blood pressure unexplained by other medical reason. moderate
3 Biochemical testing for phaeochromocytoma and paraganglioma might be considered prior to any elective surgical procedures requiring general anaesthesia in adult patients with NF1. weak
4 As in any phaeochromocytoma and paraganglioma predisposition syndrome surgery should be considered for symptomatic or biochemically active lesions. strong
5 A cortical-sparing adrenalectomy should be the preferred approach due to the risk of metachronous contralateral adrenal tumour. moderate

Note. NF1 = Neurofibromatosis type 1.