Table 7.

Guideline Recommendations for plexiform neurofibroma.

Plexiform neurofibroma
No	Recommendations	Strength
1	Clinical assessment should be by observation, palpation and neurological examination and should be performed by clinicians with NF1 expertise. Photography or video of the plexiform neurofibroma can be useful adjuncts.	moderate
2	Clinical assessment for plexiform neurofibroma should start at diagnosis or birth and should be carried out at every clinical visit.	moderate
3	Imaging by whole body MRI (WB-MRI) to monitor for plexiform neurofibromas should be performed at least at transition from childhood to adulthood to evaluate internal tumour burden as a predictor for the development of malignant peripheral nerve sheath tumour (MPNST) risk. WB-MRI assessment at higher frequency may be considered for patients at high risk for MPNST.	weak
4	The frequency of repeat imaging should be determined on an individual basis guided by the multidisciplinary team assessment of the level of risk for the individual. Increased assessment may be considered for patients with high risk for MPNST. In absence of internal neurofibromas at WB-MRI at transition age to adulthood clinical assessment only is required.	moderate
5	Clinical monitoring of plexiform neurofibromas should start when first detected and repeated during each visit.	moderate
6	Symptomatic plexiform neurofibromas require increased monitoring at shorter intervals for ANNUBP/MPNST. With careful judgement, it is appropriate to use 18FDG PET MRI (preferred) or 18FDG PET CT (if 18FDG PET MRI is not available) combined with clinical assessment and MRI in the diagnostic process, prior to discussing the need for biopsy.	moderate
7	For symptomatic plexiform neurofibromaa, surgery is the only treatment that can potentially cure the tumour. Plexiform neurofibroma surgery should be considered.	moderate
8	If part of standard national care, MEK-inhibitors may be considered as treatment option for symptomatic plexiform neurofibromaa, and inoperable symptomatic plexiform neurofibromas.	moderate
9	Management of plexiform neurofibroma should be decided upon and performed by a multidisciplinary team with expertise in NF1.	weak
10	Given the burden of having a potential risk of malignancy and visible manifestation in patients with NF1 with plexiform neurofibroma, people with plexiform neurofibromas should be offered psychological support in decisions of management (please see recommendations in the psychosocial needs, Table 15).	weak

NF1 = Neurofibromatosis type 1; WB-MRI = whole-body magnetic resonance imaging; MPNST = malignant peripheral nerve sheath tumour; ANNUBP = Atypical neurofibromateous neoplasm of uncertain biologic potential; ¹⁸FDG PET MRI = 18F-fluorodeoxyglucose positron emission tomography magnetic resonance imaging; ¹⁸FDG PET CT = 18F-fluorodeoxyglucose positron emission tomography computed tomography; MRI = magnetic resonance imaging; MEK = mitogen-activated protein kinase.

^asymptomatic plexiform neurofibromas are: persistent pain not responsive to treatment in regional pain centre, disfigurement, functional deficit or potential deficit including neurological deficit, bladder, bowel, respiratory or swallowing problems or haemorrhage.