Disease Directory Neurofibromatosis Type 1
Neurological

Neurofibromatosis Type 1

Also known as: NF1, von Recklinghausen disease, peripheral neurofibromatosis, NF1 haploinsufficiency

Prevalence

1-5 per 10,000 (Orphanet)

Onset

Childhood

Type

Autosomal dominant genetic

Gene

NF1

About Neurofibromatosis Type 1

Neurofibromatosis type 1 (NF1) is one of the most common single-gene neurological disorders, caused by mutations in the NF1 tumor suppressor gene encoding neurofibromin, a Ras-GTPase activating protein. Clinical features include cafe-au-lait macules, cutaneous and plexiform neurofibromas, Lisch nodules (iris hamartomas), optic pathway gliomas, and learning disabilities. Malignant peripheral nerve sheath tumors (MPNSTs) are a serious complication. Selumetinib (Koselugo) is approved for plexiform neurofibromas.

Common Clinical Features

Cafe-au-lait macules (6 or more) Cutaneous and plexiform neurofibromas Lisch nodules Optic pathway glioma Learning disabilities Freckling in axilla/groin Skeletal abnormalities

Clinical Trial Eligibility Tips

What to know before applying to Neurofibromatosis Type 1 trials.

Selumetinib (Koselugo) is approved for plexiform neurofibromas — prior MEK inhibitor therapy must be disclosed for trial eligibility

Tumor volumetric MRI measurement of target plexiform neurofibromas is the standard eligibility and efficacy endpoint

Optic glioma trials require ophthalmological assessment including visual acuity and visual field testing at baseline

NF1 genotype class (truncating vs. missense vs. whole-gene deletion) can affect phenotype severity and should be documented

Patient Resources

Patient Organization

Children's Tumor Foundation

Visit website ↗

Natural History Registry

NF Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:636)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Neurofibromatosis Type 1 trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Neurofibromatosis Type 1. Updated daily.

Related Rare Diseases