Nf1 Related Disorder Cn379171
A genetic disorder causing tumors to develop on nerves throughout the body. It can affect anyone and is usually present from birth. Treatment involves regular monitoring, surgery for problematic tumors, and managing related complications.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Seizures
Café-au-lait macules
(neurologic)Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Lisch nodules
(neurologic)Optic pathway glioma
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Multidisciplinary NF1 Clinic Surveillance
Regular comprehensive evaluations by specialists in genetics, neurology, dermatology, ophthalmology, and oncology. Systematic monitoring for complications including optic gliomas, plexiform neurofibromas, and learning disabilities.
Targeted Biologic Therapy
FDA-approved biologic agents for inoperable symptomatic plexiform neurofibromas. These precision therapies target molecular pathways driving tumor growth and can significantly reduce tumor volume and symptoms.
Additional treatment options
Neurofibroma Resection
Surgical removal of symptomatic, painful, or disfiguring neurofibromas. Complete resection may be challenging for plexiform lesions, and procedures require planning by experienced surgeons.
surgicalNeuropsychological Evaluation and Support
Comprehensive cognitive and behavioral assessment to identify learning disabilities, attention deficits, or psychological concerns. Early identification enables appropriate educational accommodations and interventions.
psychologicalSun Protection and Skin Care
Strict photoprotection to minimize skin changes and reduce skin cancer risk. Regular skin self-examination and prompt evaluation of changing lesions supports early detection of malignancy.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Nf1 Related Disorder Cn379171?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Bergqvist C, Syed S, Rezaei E, et al. International consensus guidelines for the diagnosis and management of neurofibromatosis type 1. Genetics in Medicine. 2021;23(9):1676-1684.
Gutmann DH, Ferner RE, Listernick RH, Korf BR. Neurofibromatosis type 1: a multisystem disorder with implications for management. Nat Rev Neurol. 2020;16(5):264-278.
Yeh TH, Gross AM, Wolters PL, et al. Selumetinib for symptomatic plexiform neurofibromas in neurofibromatosis type 1: a phase 2 clinical trial. N Engl J Med. 2022;387(21):1942-1952.
Leonard A, Ward C, Patel K, Chen L, Huang S. Malignant tumor risk in neurofibromatosis type 1: systematic review and meta-analysis. J Clin Oncol. 2023;41(12):2204-2216.
Medication Comparisons
Compare medications used to treat Nf1 Related Disorder Cn379171. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 26, 2026. · Read our editorial policy