Schwannoma

Información en español Title

Other Names:

Neurilemoma; Neurilemmoma; Neurolemmoma; Neurilemoma; Neurilemmoma; Neurolemmoma; Peripheral fibroblastoma; Benign schwannoma See More

Subtypes:

Acoustic neuroma

Summary Summary

A schwannoma is a tumor of the peripheral nervous system or nerve root. A schwannoma develops from cells called Schwann cells, a type of cell that wraps itself around peripheral nerves and provides protection and support. Schwannomas are almost always benign (not cancerous), but rarely, may become cancerous (a malignant schwannoma).^[1]^[2] Symptoms of a schwannoma may be vague and will vary depending on its location and size, but may include a lump or bump that can be seen or felt, pain, muscle weakness, tingling, numbness, hearing problems, and/or facial paralysis.^[1]^[2]^[3] Sometimes schwannomas do not cause any symptoms.^[1]

Schwannomas usually develop in otherwise healthy people for unknown reasons.^[2] In some cases, a schwannoma is caused by a genetic disorder such as neurofibromatosis 2 (NF2), schwannomatosis, or Carney complex.^[1]^[2]^[4] People with these genetic disorders usually have more than one schwannoma.^[1]^[2]

Schwannomas may be diagnosed using imaging studies. A biopsy may confirm the diagnosis.^[1]^[2] Treatment of benign schwannomas may involve surgery to remove the tumor.^[2] Treatment of malignant schwannomas may include both surgery and radiation therapy.^[5]

Last updated: 12/6/2017

Symptoms Symptoms

Schwannomas may grow slowly and may be present for months or years without causing symptoms.^[2] Therefore, some people may never experience any symptoms.^[1] Others may experience radiating pain, muscle weakness, tingling, a "pins and needles" sensation, or numbness, but symptoms vary depending on the peripheral nerve which is affected.^[1] The following are examples of symptoms that may occur due to a schwannoma in a specific nerve or area of the body:

Vestibular nerve (the nerve connecting the ear and brain) - hearing loss, dizziness, balance problems, and/or ringing or buzzing in the ear.^[6]
Facial nerve - facial paralysis, swallowing problems, difficulty moving the eye, facial pain, and/or loss of sense of taste.^[3]
Spinal nerve root - symptoms similar to a herniated disk such as radiating arm or leg pain, numbness, tingling, and muscle weakness.^[2]^[7]
Arm or leg nerve - localized pain, "pins and needles" sensation, carpal tunnel syndrome (if on a wrist nerve), and tarsal tunnel syndrome (if on an ankle nerve).^[2]

Last updated: 12/7/2017

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Abnormality of peripheral nervous system electrophysiology		0030177
Abnormality of the temporal bone		0009911
Peripheral Schwannoma		0009593
Scleral schwannoma		0100011
Vestibular Schwannoma		0009588
30%-79% of people have these symptoms
Allodynia		0012533
Facial palsy	Bell's palsy	0010628
Hearing abnormality	Abnormal hearing	0000364
Vertigo	Dizzy spell	0002321
5%-29% of people have these symptoms
Abnormal parotid gland morphology		0000197
Abnormality of esophagus morphology		0002031
Abnormality of fibula morphology	Abnormality of the calf bone	0002991
Abnormality of the adrenal glands	Adrenal abnormalities	0000834
Abnormality of the breast		0000769
Abnormality of the larynx		0001600
Abnormality of the liver	Abnormal liver Liver abnormality [ more ]	0001392
Abnormality of the twelfth cranial nerve		0010826
Acute episodes of neuropathic symptoms		0003489
Intestinal polyposis		0200008
Morphological abnormality of the central nervous system		0002011
Nasal polyposis		0100582

Showing of 21 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 10/1/2018

Do you have updated information on this disease? We want to hear from you.

Cause Cause

In most cases, it is not known why a schwannoma develops. In some cases, a schwannoma develops in association with an underlying genetic disorder such as neurofibromatosis 2 (NF2), schwannomatosis, or Carney complex. These disorders may cause multiple tumors to develop.^[1]^[2]^[6] When a schwannoma is a feature of a genetic disorder, it is caused by a genetic mutation that causes an increased risk for tumor growth.^[4]^[8]

Schwannomas develop from the Schwann cells which normally form a protective lining around most of the nerves of the peripheral nervous system and also the nerve root. The peripheral nervous system carries signals from the brain and spinal cord (central nervous system) to the muscles and tissues of the body. The nerve root is the first part of nerve leaving the spinal cord and then becomes a peripheral nerve. Schwann cells provide support by wrapping around nerves and nerve roots but also produce the fatty insulation called myelin that surrounds nerves and helps nerve signals travel fast. When a schwannoma grows large enough, it may begin compressing or putting a squeezing pressure on the nerve. It also damages the myelin in that area of the nerve. Together the compression of the nerve and myelin damage cause the symptoms related to schwannomas.^[2]^[6]

Last updated: 12/7/2017

Inheritance Inheritance

Most schwannomas are not inherited. The vast majority occur by chance and as a single tumor.^[9]

In some cases, a person develops a schwannoma (or multiple schwannomas) due to having an underlying genetic disorder such as neurofibromatosis 2 (NF2), schwannomatosis, or Carney complex.^[1]^[2]^[4] These disorders are inherited in an autosomal dominant manner.^[8]^[4] This means that having a change (mutation) in only one copy of the responsible gene in each cell is enough to cause features of the condition.

In some cases, a person with an autosomal dominant disorder inherits the mutation from a parent with the disorder. In other cases, the mutation occurs for the first time in a person with no family history of the condition. This is called a de novo mutation.

When a person with a mutation that causes an autosomal dominant disorder has children, each of his/her children has a 50% (1 in 2) chance to inherit that mutation.

Last updated: 12/7/2017

Diagnosis Diagnosis

A schwannoma may be difficult to diagnosis at first because, depending on its location, the symptoms it causes may be similar to the symptoms caused by other health problems. The following tests may be used to rule out other causes of the symptoms and confirm the diagnosis:^[1]^[2]^[10]

X-ray
Ultrasound
CT scan
Magnetic resonance imaging (MRI) - MRI can help determine if a tumor is on the outside of a nerve or part of a nerve and if it involves other nearby structures
Biopsy of the tumor to confirm the diagnosis - a biopsy involves removing a small sample of the schwannoma to be examined under the microscope

In some cases, a schwannoma is discovered incidentally (by chance) when a person has imaging studies for another reason.^[1]

Last updated: 12/7/2017

Treatment Treatment

Treatment for a schwannoma may depend on the location of the tumor, severity of symptoms, and whether the tumor is benign or malignant (cancerous).^[6] A benign schwannoma causing symptoms or affecting a person's appearance is typically treated with surgery to remove as much as the tumor as possible, while keeping the affected nerve intact. It is often possible to remove the entire tumor. Surgery usually quickly relieves the related symptoms, although if muscle weakness was present before surgery, the muscle may not return to full strength.^[2]^[6]^[10]

Treatment for a malignant schwannoma may involve both surgery and radiation therapy.^[2]^[6]^[10]

Last updated: 12/7/2017

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Schwannoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

American Brain Tumor Association
8550 W. Bryn Mawr Ave, Ste 550
Chicago, IL 60631
Toll-free: 1-800-886-2282
Telephone: +1-773-577-8750
Fax: +1-773-577-8738
E-mail: info@abta.org
Website: https://www.abta.org/

Social Networking Websites

Visit the Schwannoma Survivors & Schwannoma Fighters group on Facebook.

Organizations Providing General Support

American Cancer Society
250 Williams Street NW
Atlanta, GA 30329
Toll-free: 800-227-2345
Website: http://www.cancer.org

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The American Brain Tumor Association has an information page on Schwannoma. Click on the link to view this information page.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Schwannoma. Click on the link to view a sample search on this topic.

News & Events News & Events

News

NCATS Rare Diseases Are Not Rare! Challenge
October 9, 2018
The NIH Undiagnosed Diseases Network Expands
September 26, 2018

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

I had a schwannoma tumor removed from my stomach. It was a cellular type of schwannoma, and it was benign. What is the difference between this type of schwannoma and other types? Will it grow back? Should I be tested for this condition for the rest of my life? See answer
Is it possible to have a schwannoma again after surgery? See answer
I had a schwannoma removed from my arm. I now have numbness in the area and tingling in my finger. Will these sensations be permanent? And could a previous trauma cause a schwannoma? See answer
Can a schwanomma cause vertigo if it is near an ear? See answer
I have a fairly large schwannoma/myxoma tumor in my right arm in the tricept. I would like to know if I'm likely to have other such tumors in other parts of my body. See answer