Schwannomatosis: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 2, 2026.
What kind of doctor treats Schwannomatosis?
A primary care doctor is often the first stop, and care usually also involves specialists such as a neurologist, a neurosurgeon, or a geneticist. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What it is
Schwannomatosis is a rare condition that causes noncancerous tumors called schwannomas to grow on the covering of nerves. These tumors grow from Schwann cells, which wrap around nerves and help them send signals. Most people develop tumors in adulthood, often after age 30.
Symptoms
The most common symptom is pain, which can be long lasting and often feels worse than the size of the tumors would suggest. Some people also have numbness, tingling, or muscle weakness near a tumor.
Causes and who gets it
Schwannomatosis is a genetic condition. Changes in two genes, SMARCB1 and LZTR1, are linked to most cases. Some people inherit the gene change; others are the first in their family to have it. It affects about 1 in 25,000 to 30,000 people.
How doctors diagnose it
Doctors usually start with a physical exam and symptom history. An MRI can show the tumors and where they sit. A genetic test, done with blood or saliva, looks for the linked gene changes.
Treatment
There is no cure, but treatment can ease symptoms. Pain medicines, including some drugs originally made for seizures or depression, often help. Physical therapy can help with strength and movement. Surgery may be an option if a tumor presses on a nerve or the spinal cord, though tumors can grow back or new ones can appear.
When to see a doctor
See a doctor for ongoing pain, numbness, tingling, or muscle weakness that does not go away. Seek care quickly for sudden weakness, trouble with balance, or changes in vision or hearing.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance; a concierge doctor usually charges more and often works alongside insurance. Either can order MRI scans, adjust pain medicines, and coordinate care, referring to specialists when needed.
Common questions
Is schwannomatosis the same as neurofibromatosis?
It is related but not the same. Schwannomatosis was once considered a form of neurofibromatosis, a group of conditions that cause nerve tumors. It is now seen as its own condition with different gene changes. The tumors in schwannomatosis are schwannomas, which grow from the nerve covering rather than from the nerve fibers themselves.
Are the tumors cancerous?
In most cases, no. The tumors are almost always benign, which means they are not cancer and do not spread to other parts of the body. Rarely, a tumor can become cancerous, so doctors monitor them over time.
Can schwannomatosis be passed to children?
Yes, it can be inherited from a parent who has the gene change. A child of a parent with the condition has a 50 percent chance of inheriting it. Some people are the first in their family to develop the gene change.
Do all tumors need surgery?
No. Many tumors are watched with regular scans instead of being removed. Surgery is usually considered when a tumor causes severe pain, weakness, or pressure on the spinal cord.
Sources
- NIH Genetic and Rare Diseases Information Center - Schwannomatosis
- MedlinePlus Genetics - Schwannomatosis
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
