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Three separate genetic conditions that cause tumours to grow on nerves. Most are benign, and many need watching rather than treating — but knowing which type you have determines what to watch for and when to act.
Neurofibromatosis is a group of genetic conditions in which tumours grow on nerve tissue — in the brain, the spinal cord, and the nerves running between them and the rest of the body. Most of these tumours are benign, though a small proportion become malignant.
Three conditions carry the name, and they are genuinely different. They involve different genes, appear at different ages, and cause different problems. Grouping them under one word is a historical accident rather than a clinical judgement, and it is the main source of confusion for people newly diagnosed.
The commonest type, also called von Recklinghausen disease. Usually recognised in childhood, often at or shortly after birth.
Severity varies widely. Many people with NF1 have a mild form and lead entirely ordinary lives.
Much less common than NF1, and defined by benign, slow-growing tumours on the hearing and balance nerves of both ears — acoustic neuromas, also called vestibular schwannomas.
This is the type most squarely in neurosurgical territory. Acoustic neuromas are tumours of the nerve between the inner ear and the brain, and decisions about monitoring, surgery or radiation are neurosurgical ones.
The rarest of the three. Tumours develop on cranial, spinal and peripheral nerves, but rarely on both hearing nerves — which is what distinguishes it from NF2.
Pain rather than hearing loss is the defining problem here, and it is often what brings people in.
Each type involves a different gene, and in each case the gene normally works to suppress tumour growth.
Where it is inherited, each child of an affected parent has a 50% chance of inheriting the gene — an autosomal dominant pattern. That figure applies to each pregnancy independently, and it is the reason genetic counselling is part of the picture for families rather than an afterthought.
Assessment begins with your history, any family history, and an examination covering the skin, the eyes, the spine and neurological function. From there:
Neurofibromatosis is a lifelong condition, and its overall care is best coordinated by a genetics service working with several specialties. No single neurofibromatosis specialist covers all of it, and it would be misleading to present ourselves as that coordinating service.
What this clinic treats is the specific problems the condition produces — and between the surgeons here, that covers most of them.
Acoustic neuromas in NF2, and schwannomas on the spinal and peripheral nerves, are neurosurgical. Decisions turn on size, growth rate, position and what the tumour is pressing on — and often the right answer is monitoring rather than operating. Dr Mathew Tung's practice includes microscopic brain tumour surgery.
Chronic nerve pain is the dominant symptom in schwannomatosis and common in NF1. Where medication has not controlled it, treatment can be directed at the nerves themselves — nerve blocks, pulsed radiofrequency and neuromodulation.
Scoliosis is one of the commoner complications of NF1, and bone changes including osteoporosis are part of the picture. Dr Lim Heng Hing is an orthopaedic spine surgeon, working from the same clinic — which means the spine and the nerve tumours can be assessed together rather than in separate places.
For many people the right course is regular review and imaging rather than intervention — watching for growth, for hearing change, for a tumour beginning to press on something. That is an active decision, not an absence of one.
Mild NF1, NF2 and schwannomatosis often need no treatment beyond observation, regular review and managing symptoms as they arise. Children diagnosed with neurofibromatosis are monitored for complications — hearing problems, bone changes, and changes in skin lesions.
Where a tumour causes significant pain, affects neurological function, presses on a nearby structure, or shows rapid growth on imaging, removing it surgically or treating it with radiation becomes the question. Which of those applies depends on where the tumour is and what it is doing.
Usually not. The great majority of tumours in all three types are benign. A small proportion can become malignant, which is one of the reasons regular monitoring matters — change is easier to act on when it is noticed early.
Around half of NF1 cases arise from a spontaneous mutation with no family history at all, and the same happens in NF2. It is not something you did or inherited; the gene changed on its own.
Where the condition is inherited, each child has a 50% chance, independently for each pregnancy. Genetic counselling gives you a proper assessment of your own situation rather than a general figure.
No, and removing them all would cause more harm than good. Surgery is considered where a tumour is causing pain, affecting function, pressing on something, or growing quickly. Many are simply watched.
Both cause schwannomas, but NF2 characteristically affects the hearing and balance nerves of both ears, causing progressive hearing loss. Schwannomatosis rarely does, and pain is its dominant symptom instead.
No. You can book directly. Bring any imaging, genetic test results and previous specialist letters — with a lifelong condition, the history matters as much as the current scan.
Neuropathic pain · Scoliosis · Osteoporosis · Dizziness and vertigo · Headaches (all types) · Neuromodulation · Neurosurgery
Bring your previous imaging as well as the latest. With a condition measured in years, what has changed matters more than any single scan.
