The evidence supporting these management guidelines is provided by expert consensus opinion.
Some types of cancer in NF1 are associated with poorer survival when compared with general population controls, particularly in those diagnosed at age <40 years.r Therefore, concerning symptoms should be investigated promptly.
The type, timing and degree of intervention/ surgery, chemotherapy or radiotherapy treatment of NF1 patients (if any) should be carefully made by a multidisciplinary team with experience in managing NF1, the lifelong implications of the condition, its treatments and the complications that may result from treatment.rr Radiotherapy is avoided where possible due to high risk of secondary tumours.r
Other possible NF1 associated cancers include juvenile myelomonocytic leukaemia, thyroid cancer, malignant fibrous histiocytomas and rhabdomyosarcoma.rr
Optic nerve pathway gliomas
Most optic nerve gliomas occur around 3-5 years of age, and do not require intervention.rr
The effectiveness of breast cancer screening in women with NF1 is still unclear and data about the utility of screening programs is mostly extrapolated from cancer screening studies in other groups at increased risk of breast cancer.r