About Neurofibromatosis Type 1
Neurofibromatosis type 1 (NF1) is caused by loss-of-function mutations in the NF1 gene, which normally produces neurofibromin, a protein that regulates the RAS-MAPK cell signaling pathway. When neurofibromin is absent or defective, this pathway becomes overactive, driving uncontrolled cell growth and tumor formation.
The hallmark features are cafe-au-lait macules (present in nearly all patients by age 1), cutaneous neurofibromas (which develop during puberty and adulthood), and plexiform neurofibromas (congenital tumors that can grow to significant size and cause pain, disfigurement, and functional impairment). About 15 to 30% of patients with plexiform neurofibromas develop symptomatic tumors requiring treatment.
NF1 carries an 8 to 13% lifetime risk of malignant peripheral nerve sheath tumor (MPNST), a serious complication. Other features include learning disabilities (affecting 50 to 75% of children), skeletal abnormalities, cardiovascular complications, and optic pathway gliomas in approximately 15% of children. Two MEK inhibitors, selumetinib (Koselugo) and mirdametinib (Gomekli), are now FDA-approved for symptomatic plexiform neurofibromas, marking a major shift from surgery as the only option.
Common Symptoms of Neurofibromatosis Type 1
Recognizing the signs of Neurofibromatosis Type 1 early can lead to faster diagnosis and better outcomes. Symptoms may vary in severity from person to person. If you or a loved one are experiencing any of the following, consider speaking with a specialist.
- Cafe-au-lait spots (flat, light brown patches on the skin appearing in early childhood)
- Neurofibromas (soft bumps on or under the skin) that increase in number with age
- Plexiform neurofibromas (larger tumors growing along nerve bundles causing pain and disfigurement)
- Freckling in the armpits or groin area (Crowe sign)
- Optic pathway gliomas affecting vision, typically in children under 7
- Bone abnormalities including scoliosis, tibial bowing, and sphenoid wing dysplasia
Who Neurofibromatosis Type 1 Affects
Present from birth with autosomal dominant inheritance. About half of cases arise from new (de novo) mutations with no family history. Affects males and females equally across all ethnicities.
Symptoms typically become apparent in childhood, though plexiform neurofibromas and malignant transformation risk increase with age.
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FDA-Approved Treatments for Neurofibromatosis Type 1
There are currently 2 FDA-approved medications for Neurofibromatosis Type 1. These therapies represent the current standard of care and may be used alongside or compared against investigational treatments in active clinical trials.
Source: openFDA drug labeling data. This list may not include all treatments. Always consult your doctor.
Help Paying for Neurofibromatosis Type 1 Treatment
Charity funds and drugmaker programs for Neurofibromatosis Type 1, checked at the source. Pick your insurance to see what fits.
- From a charity · NORD RareCareNeurofibromatosis Type 1 Medical Assistance fundOpen
Pays for: Medical and medication costs.
The foundation says: “Accepting Applications” - From a charity · NORD RareCareNeurofibromatosis Type 1 Premium Copay Assistance fundOpen
Pays for: Insurance premiums and copays.
The foundation says: “Accepting new applications and re-enrollments for current year” - From a charity · The Assistance FundNeurofibromatosis fundOpen
Pays for: Copays, coinsurance, deductibles and other health-related expenses.
The foundation says: “OPEN — Accepting New Patients. TAF is currently accepting new patient enrollments for this program.” - From a charity · TotalAssist (formerly PAN Foundation)Neurofibromatosis fundOpen
Pays for: Out-of-pocket costs for approved medications, up to $6,400 per year. Requires health insurance (any kind).
Side Effect Explorer
Real-world side effect reports from the FDA Adverse Event Reporting System (FAERS). Includes both FDA-approved drugs and investigational therapies from active clinical trials. Click any drug to see what patients reported.
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Genetic Testing
Genetic testing can confirm a diagnosis, guide treatment decisions, and identify family members who may be at risk.
The NF1 gene page lists every condition Orphanet links to the gene and the open trials that name it.
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Trusted Neurofibromatosis Type 1 Resources
Reputable organizations and medical references for learning more about Neurofibromatosis Type 1, including disease registries, foundation resources, and clinical guidelines.
