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.