What percentage of NF1 patients develop malignant peripheral nerve sheath tumors?

MPNST occurs in 8-13% of NF1 patients (vs 0.001% general population); lifetime risk is 10%; suspicious features include rapid growth, pain, hardness, and heterogeneity on MRI.

What is the eligibility for selumetinib in NF1?

Selumetinib (Koselugo) is approved for children ≥2 years with NF1 and symptomatic inoperable plexiform neurofibromas; ≥20% tumor volume reduction in trials is the response threshold.

What diagnostic criteria define NF1?

2 or more of: ≥6 cafe-au-lait macules >5mm (prepubertal)/>15mm (postpubertal), ≥2 neurofibromas or 1 plexiform, axillary/inguinal freckling, optic glioma, ≥2 Lisch nodules, bony dysplasia, first-degree relative with NF1.

What optic glioma features require treatment?

Progressive visual loss, increasing proptosis, diencephalic syndrome with failure to thrive, or symptomatic hydrocephalus warrant treatment; chemotherapy (carboplatin/vincristine) is first-line.

How is learning disability managed in NF1?

Cognitive impairment occurs in 50-80% of NF1; specific learning disabilities, ADHD, and processing speed deficits are most common; educational support and ADHD treatment (stimulants are safe in NF1) are recommended.

Neurofibromatosis Type 1 Disease Burden Calculator

Calculates NF1 disease burden including plexiform neurofibroma tumor volume, MPNST risk assessment, optic glioma monitoring parameters, and selumetinib eligibility criteria.

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Frequently Asked Questions

What percentage of NF1 patients develop malignant peripheral nerve sheath tumors?

What is the eligibility for selumetinib in NF1?

What diagnostic criteria define NF1?

What optic glioma features require treatment?

How is learning disability managed in NF1?