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CION Cancer Clinics
Positive for NF2, SMARCB1 or LZTR1: what happens next | CION Cancer Clinics
A positive result in NF2, SMARCB1 or LZTR1 does not need action today. It means a planned set of checks, a team who knows your gene, and a careful conversation about relatives. What happens next depends on which gene is involved, and on whether you already have tumours. This page walks through the first steps in order, what changes and what stays the same. At CION Cancer Clinics, our oncologists explain what a gene result means for you and your family, and plan the checks that follow.
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Medically reviewed by Dr. Naresh GunduConsultant Medical Oncologist · MBBS, DNB (Internal Medicine), DM (Medical Oncology, AIIMS) · last reviewed September 2026, next review due September 2027
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On this page
- What should happen after a positive NF2, SMARCB1 or LZTR1 result?
- Which result do you have, and what does it usually lead to?
- What are the first steps, one at a time?
- The words you will meet, in plain language
- What changes now, and what does not
- What this page cannot tell you
- Four things people fear after a positive result
- Common questions after a positive result
The short answer
What should happen after a positive NF2, SMARCB1 or LZTR1 result?
Nothing needs to happen today. A positive result is a reason to plan, not an emergency. The next steps are a result appointment with a counsellor, a first set of baseline checks, and a named team to follow you. Telling relatives comes after that, at a pace you choose.
If you already have tumours
The result explains why they formed, and it changes how treatment is chosen. In NF2, radiotherapy is weighed more carefully than usual, and a drug that starves tumours of new blood vessels is sometimes used to slow growing tumours on the hearing nerves. Your team will explain which of these applies to you.
If you are well and were tested because of a relative
For NF2, a baseline MRI and hearing test set the starting point, so later scans have something to be compared with. For SMARCB1 and LZTR1, many carriers never develop a tumour. Checks are usually lighter and often begin in adult life.
A positive result is a statement about risk. It is not a diagnosis of anything new.Four different results
Which result do you have, and what does it usually lead to?
The three genes share a family of tumours, but a positive result in each one means something quite different.
NF2, in every cell
The fault is present from birth throughout the body. Tumours on both hearing nerves are the hallmark, often with growths on the brain's lining or the spine. A lifelong plan of scans and hearing tests follows.
NF2, mosaic
The fault is present in only some cells, often because it arose early in development. The illness can be milder or limited to one part of the body. Your children face a lower chance of inheriting it.
SMARCB1
Linked with schwannomatosis, where tumours grow on nerves in the spine, arms and legs, and sometimes with growths on the brain's lining. Pain is often the first and main problem.
LZTR1
Also linked with schwannomatosis, but many carriers stay well.
What usually follows
- Attention to new pain or lumps along a nerve
- A scan when symptoms suggest one
- Occasional hearing-nerve tumours, usually on one side
Not sure whether this applies to you?
Ask an oncologistIn order
What are the first steps, one at a time?
The result appointment
A counsellor or clinical geneticist explains the exact fault, how certain the laboratory is about it and what it means for you. Bring a family member and write your questions down beforehand.
Baseline checks
For NF2, an MRI of the brain and spine, a hearing test and an eye examination. For SMARCB1 or LZTR1, the team decides from your symptoms whether a scan is needed now.
A named team
NF2 care is shared between neurosurgery, ENT, audiology and eye specialists. Ask who is coordinating, and who you call if a new symptom appears between visits.
Telling the family
Your counsellor can give you a family letter. It names the exact fault, so relatives can be tested for it without starting again.
On your report and scans
The words you will meet, in plain language
- NF2-related schwannomatosis
- The current name for the condition once called neurofibromatosis type 2. Both names describe the same thing.
- Schwannoma
- A non-cancerous growth from the coating that wraps around a nerve. It causes trouble by pressing on the nerve or on nearby tissue.
- Vestibular schwannoma
- A schwannoma on the nerve for hearing and balance. Some reports call it an acoustic neuroma.
- Meningioma
- A usually non-cancerous growth from the thin lining around the brain or spinal cord.
- Ependymoma
- A growth inside the spinal cord or brain. In NF2 these are usually slow and are often simply watched.
- Pathogenic variant
- A change in the gene known to stop it working. This is what the report means by a positive result.
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Side by side
What changes now, and what does not
What changes
What stays the same
You follow a planned schedule of checks
You can keep working, studying and travelling
Radiotherapy is weighed more carefully
Most tumours remain non-cancerous
New symptoms are assessed sooner
Nothing needs to happen today
Family planning can be discussed
Marriage and children remain your choice
Being straight with you
What this page cannot tell you
It cannot tell you how your own condition will behave. NF2 varies widely, even between a parent and child with the same fault. Some people have a few slow tumours for decades. Others need treatment young. The pattern of your scans over time is the best guide, and only your team can read it.
It cannot interpret your variant
The type of fault can hint at how NF2 may behave, but the link is not exact. What your specific variant means is a question for the counsellor who ordered the test. If a variant is labelled uncertain, it is not a positive result and should not start a surveillance plan.
Who this does not apply to
This page is not for someone with a single hearing-nerve tumour and no fault found. Most people in that situation do not have NF2. It is also not for SMARCB1 faults found in a baby with a rhabdoid tumour, which is a different condition with its own plan.
Keep a folder of every report and scan disc. It will save you repeating tests if you change teams or cities.Commonly believed
Four things people fear after a positive result
"NF2 means I will go completely deaf soon."
Many people with NF2 lose some hearing over time, but the pace varies widely. Careful monitoring and well-timed treatment aim to keep useful hearing for as long as possible. Hearing aids and implants help when hearing does fall.
"These tumours are cancer and will spread."
NF2 and schwannomatosis tumours are almost always non-cancerous and do not spread through the body. They cause harm by pressing on nerves. A change to cancer is rare.
"Every tumour has to be operated on straight away."
Many tumours are watched for years. Treatment is timed by growth, symptoms and hearing, because each operation near a nerve carries its own risks. Waiting under close watch is a real, active choice.
"An LZTR1 result means the same as NF2."
It does not. LZTR1 faults usually cause tumours on nerves outside the head, and many carriers never have a tumour. The plan is lighter and starts later.
Questions we are asked
Common questions after a positive result
Is there a medicine for NF2?
There is no medicine that removes the fault. A drug that blocks new blood vessels is sometimes used to slow growing tumours on the hearing nerves and help protect hearing. Newer drugs are being studied. Your team will say whether any are suitable, and whether a trial could be an option.
Does NF2 raise my risk of cancer?
The tumours in NF2 are almost always non-cancerous. A change to cancer is rare, and has been linked in some people with earlier radiotherapy. That is one reason radiotherapy is used carefully. NF2 is looked after partly by tumour teams because the growths still need expert watching.
Can I keep working and driving?
Most people keep working and living normally for long periods. Driving depends on your balance, vision and any seizures, and your doctor can advise. Tell your team about any job that relies on hearing or balance, so they can plan around it.
Can I still marry and have children?
Yes. Each child has a chance of inheriting the fault, and there are options such as testing embryos during IVF if you want to avoid that. A counsellor can help you think about what to tell a partner's family, and when. The choice stays with you.
Should I tell my insurer?
India has no dedicated law on genetic discrimination in insurance, and forms differ in what they ask. Answer every question on a form honestly. Talk to your counsellor before you apply for new cover, because the wording of the question matters.
Where will I be looked after?
Usually by several teams: neurosurgery, ENT and audiology, eye care and genetics. Scans and hearing tests can be done in Hyderabad. The genetics team at CION can explain the result and help you reach specialists who see NF2 regularly.
The result came from a wide panel test and nobody has tumours. What now?
This happens, especially with LZTR1. A fault found by chance in a family with no tumours often carries a low risk. Do not start scans on your own. Ask the counsellor who ordered the test whether any checks are needed at all.
How do I tell my children?
Simply, honestly and in stages that suit their age. For NF2, younger children will need testing, so parents often explain it as a check to keep them healthy. Your counsellor can suggest words, and can meet older children with you.
Your Specialists
Meet CION's oncologists. Bring your family history or genetic report to them.
Our medical oncologists see people with a strong family history of cancer, arrange genetic counselling and testing where it fits, and plan the checks that follow.
Medical Oncologist
Dr. C. Raghavendra Reddy
MBBS(Gold Medal), DNB(General Medicine), DM(Medical Oncology)(Gold Medal)
Medical Oncologist
Dr. Bharati Devi Gorantla
MBBS, MD(General Medicine), DM(Medical Oncology)(Adyar,Chennai), ECMO, MRCP SCE(UK)
Medical Oncologist
Dr. Owais Mohammed
MBBS, MD (General Medicine), DrNB (Medical Oncology), ECMO, MRCP SCE (Medical Oncology) (UK)
Medical Oncologist
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Sources
- GeneReviews (NCBI Bookshelf) — NF2-Related Schwannomatosis
- MedlinePlus Genetics — NF2 gene
- MedlinePlus Genetics — Schwannomatosis
- NHS — Neurofibromatosis type 2
This page is general information, not a prescription. Do not change or stop any treatment based on what you read here. If anything is worrying you, contact your own treating team — or call our helpline and we will help you reach the right specialist.
Keep reading
Related pages
Surveillance for NF2 and schwannomatosis gene carriersThe checks you will have, and how oftenNF2 and schwannomatosis genes: which cancers and what riskWhich tumours each gene is linked withTesting the family for NF2 and schwannomatosis genesWho else should be offered a testHearing surveillance and NF2Protecting hearing for as long as possible
Talk to us
Just received a positive result and not sure what to do first?
Tell us which gene was found and whether you have any symptoms. We will help you understand the result and plan the first checks. One helpline serves every CION centre.