Neurofibromatosis refers to three genetic conditions — NF1, NF2, and schwannomatosis — that cause tumors to grow on nerves throughout the body. NF1 affects about 1 in 3,000 people and causes skin changes, learning differences, and benign tumors called neurofibromas. NF2 causes tumors on hearing and balance nerves, often leading to deafness. Schwannomatosis causes painful nerve tumors without the hearing loss of NF2.
What's actually going on in research
Trials are testing MEK inhibitors that shrink plexiform neurofibromas in NF1, drugs that target the hearing tumors in NF2, and treatments for the chronic pain of schwannomatosis. Researchers are studying why some neurofibromas become cancerous, how to prevent tumor growth before it causes problems, and whether drugs approved for NF1 can help people with NF2 or schwannomatosis.
MEK inhibitors for NF1
Selumetinib became the first FDA-approved drug for NF1 in 2020, shrinking inoperable plexiform neurofibromas in children. Trials are testing other MEK inhibitors and expanding treatment to adults and cutaneous neurofibromas.
NF2 hearing preservation
Studies are testing whether drugs can slow or stop the growth of vestibular schwannomas, the tumors that cause hearing loss in NF2. Some trials combine drug treatment with new surgical techniques that spare more hearing.
Pain management
Schwannomatosis causes severe nerve pain that standard painkillers often don't help. Trials are testing drugs that target specific pain pathways and combinations of medications tailored to nerve pain.
What to know before you search
Eligibility typically depends on neurofibromatosis type, tumor location and size, symptoms like pain or vision problems, age, and whether tumors are growing on recent scans.
What types of trials are currently open
- Tumor-shrinking trials — Testing drugs like MEK inhibitors to see if they can shrink existing neurofibromas or schwannomas, particularly tumors that can't be safely removed with surgery.
- Tumor-prevention trials — Testing whether drugs can stop new tumors from forming or slow the growth of small tumors before they cause symptoms.
- Hearing-preservation trials — Testing treatments for NF2 that aim to preserve hearing by controlling vestibular schwannoma growth without surgery.
- Pain trials — Testing medications and combinations for chronic nerve pain, especially in schwannomatosis where pain can be disabling.
- Natural history studies — Following people with neurofibromatosis over time to understand how tumors develop and what factors predict which tumors will grow or cause problems.
Recently added Neurofibromatosis trials
Observational Study of the Real-world Effectiveness and Safety of Selumetinib Treating Adult Patients With Neurofibromatosis Type 1 (NF1) Who Have Symptomatic, Inoperable Plexiform Neurofibroma (PN)
This is a prospective, multicentre, observational registry study with primary data collection designed to evaluate the real-world effectiveness and safety of selumetinib in adult patients with NF1 who have symptomatic, inoperable PN. A total of approximately 800 participants who initiate selumetinib treatment will be enrolled across about 45 investigational sites in China. Sites will be selected based on their diagnostic and treatment expertise in NF1-PN, research capability and sufficient patient pool. Patients who are eligible and willing to participate will be enrolled into the study. The study includes an approximately 25-month enrolment period and a 96-week observation period. Patients will be followed up until the end of the 96-weeks observation period after first dose of selumetinib, or patient death, lost to follow-up, withdrawal of consent, whichever occurs first. Patients will be followed within 96 weeks period (starting after first dose received) in the study even if selumetinib is discontinued. The study will end until the last participant has the opportunity to complete 96 weeks' assessment.
Take a daily capsule to shrink nerve tumors from neurofibromatosis type 1
This study aims to demonstrate that in subjects with symptomatic, inoperable plexiform neurofibromas associated with neurofibromatosis type 1, TQ-B3234 capsules significantly improve the objective response rate at Week 24 compared to placebo.
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