Table 5

Frequency of tumour complications occurring in 523 patients with NF1 in a review of patient genetic clinic and hospital notes carried out in NW England

TumourNoMedian (range) (y)Life time risk (%)
Plexiform tumours8015
Severe plexiform tumours of the head and neck255.9
Spinal neurofibromas1117  (1–47)5
Visceral neurofibromas1018 (14–39)3
Endocrine tumours (phaeochromocytomas)236 (25–51)1.5
Optic glioma254 (0–21)6
Astrocytoma615 (6–30)1.5
Malignant nerve sheath tumours1535 (13–53)7.5–15
Other CNS tumours625 (0–45)4
Vestibular Schwannoma00
NF related malignancy5-150 5-151 437 (0–53)15–20
  • 5-150 Including optic glioma.

  • 5-151 Six patients had two malignancies; second malignancies were therefore excluded from analysis.