The nerves that run throughout our bodies are like a network of wires that carry electrical messages. They are what carry information back and forth from the brain to the rest of the body, and from the body to the brain. Imagine that these nerve cells, their clusters, are all wrapped in special protective membranes, like the plastic sheath around a wire, to protect them and help the electrical signals travel faster. It is in these protective membranes that these so-called nerve sheath tumors sometimes develop.
What are Nerve Sheath Tumors?
Simply put, these tumors form in the protective "sheaths" that surround our nerves. This "sheath" or covering is made up of several layers:
- Schwann cells: These are the cells that wrap around the axon, the long part of the nerve cell, like insulation around a wire.
- Endoneurium: This is the connective tissue that surrounds each nerve fiber, much like the sheath around a single copper wire in a wire.
- Perineurium: This is what surrounds the bundles of nerve fibers.
Our nervous system can be divided into two parts:
- Central nervous system: This includes our brain and spinal cord.
- Peripheral nervous system: This is the network of nerves that branches out from the brain and spinal cord to all parts of the body.
Most of the time, these neurofibromas form in the peripheral nervous system .
What types of nuts are there?
There are several main types of neuromas. Let's take a look at what they are.
Schwannomas
These tumors develop in the Schwann cells mentioned earlier. About 60% of schwannomas develop in the vestibular nerve in our inner ear . This nerve helps our body maintain balance. Other schwannomas can sometimes develop under our skin, or deep inside the tissues and organs of the body. The most common places where these tumors are seen are:
- In the arms and legs
- In the head
- In the trunk (that is, the area between the shoulders and hips)
Schwannomas are a type of tumor that is usually covered by a thin layer of tissue, or "encapsulated." These tumors are usually noncancerous (benign). However, very rarely, a long-standing tumor can become cancerous (malignant).
Neurofibromas
This type of tumor involves several types of tissue called Schwann cells, endoneurium, and perineurium that cover the nerves. These usually appear as lumps under the skin, but can sometimes develop on nerves deep inside the body.
Unlike schwannomas, neurofibromas are not encapsulated. They grow inside nerve bundles. Plexiform neurofibromas are a type of tumor that spreads like a mesh, surrounding many nerve bundles and can spread into surrounding tissue.
Most neurofibromas are not cancerous. However, approximately 5% to 10% of these tumors can become cancerous . They are called malignant peripheral nerve sheath tumors (MPNST) . About half of people with these malignant tumors have already spread to other parts of the body by the time they are diagnosed.
How common is this fruit?
Noncancerous nerve sheath tumors are relatively rare.
- Schwannomas are most commonly seen in people between the ages of 50 and 60.
- Neurofibromas most often occur in people between the ages of 20 and 40.
- Plexiform neurofibromas usually develop before the age of 5.
Malignant Peripheral Nerve Sheath Tumors are a very rare type of cancer, affecting only about one in 10 million people each year.
Why do tumors like this form? What are the causes?
Genetic changes have a major impact on the development of these neurofibromas.
- The development of Schwannomas is associated with changes in the gene called NF2.
- Neurofibromas are associated with a gene called (NF1).
Most of the time, these genetic changes occur sporadically, without any apparent cause . However, a small number of schwannomas and neurofibromas are caused by a rare genetic condition called neurofibromatosis , which can run in families.
Types of Neurofibromatosis
There are three main types of this disease:
- Neurofibromatosis type 1 (NF1): People with NF1 develop large numbers of neurofibromas. They also have an increased risk of developing plexiform neuromas and malignant peripheral nerve sheath tumors (MPNST). Other conditions seen in people with NF1 include macrocephaly , scoliosis, and learning disabilities .
- Neurofibromatosis type 2 (NF2): Almost everyone with NF2 develops vestibular schwannomas in both ears before the age of 30. In addition, other types of schwannomas and tumors can also develop in their skin, eyes, and central nervous system.
- Schwannomatosis: In this condition, multiple schwannomas develop in the vestibular nerve (in both ears) rather than in the vestibular nerve.
What are the symptoms of these tumors?
Most people with neuromastoid tumors don't experience any pain or other symptoms . However, if the tumor grows large or presses on a nerve, symptoms such as:
- A lump or tumor under the skin (may be painful when pressed).
- Muscle weakness .
- Numbness .
- Aches, pains, or sharp pain .
- A tingling sensation, like being struck by electricity .
Depending on the location of the tumor, other specific symptoms may also occur. Here are a few examples:
- Sciatic nerve tumor: Pain that travels from the back down the leg (sciatica).
- A tumor on the wrist: Symptoms similar to those of Carpal Tunnel Syndrome.
- Vestibular nerve tumor: Hearing loss, tinnitus, balance problems.
Usually, if someone has a neurofibromatosis, it is a single tumor. However, in people with the previously mentioned condition neurofibromatosis, multiple tumors can be seen (often on the skin).
How do you know if you have these nuts?
Your doctor will first examine you physically and assess your overall health. This will include asking about your medical history, family medical history, and any symptoms you may be experiencing.
To further investigate a tumor that is already visible or a suspected tumor, your doctor may recommend the following tests:
- Medical imaging tests: These include MRI scans, ultrasound scans, and PET scans to see the tumor clearly.
- Biopsy: A small piece of tissue is taken from the tumor and examined under a microscope to determine what type of cells are present. This is what can tell you for sure whether the tumor is cancerous or not.
If you have multiple tumors, your doctor may also recommend genetic testing to see if you have conditions called (NF1), (NF2), and Schwannomatosis.
What are the treatments for this?
If you don't have any symptoms from a neurofibromatosis, your doctor may recommend a "watch and wait" approach. This means that you will be checked regularly to see if the tumor is growing or changing.
For symptomatic tumors, or if you want to remove the tumor for cosmetic reasons, surgery is usually the only option .
Some neurofibromas, especially those called plexiform neurofibromas, are difficult to completely remove because they grow inside the nerve and between the layers of the covering. If the tumor cannot be completely removed after surgery, your doctor will monitor you closely, as the tumor can grow back.
Treatment of schwannomas in the head
Schwannomas that form in the head, such as vestibular schwannomas, can affect the nerves that control important functions in our body. In these cases, doctors use a technique called stereotactic radiosurgery (e.g., Gamma Knife®) to prevent damage to the nerves.
This is not a traditional surgery that involves making an incision. It involves sending a very precisely targeted beam of radiation through the skin to destroy or shrink the tumor.
Treatment of cancerous peripheral nerve sheath tumors
In addition to surgery, cancer treatments such as radiation therapy and chemotherapy can also be used to treat malignant peripheral nerve sheath tumors.
What are the possible complications of surgery?
As with any surgery, there are several common complications that can occur with these surgeries:
- Bleeding
- Wound infection
- Pain
- Scarring
Additionally, nerve surgery carries a risk of nerve damage and permanent disability . Your medical team will help you manage any long-term disability that may arise after surgery. Various treatments (physical therapy, occupational therapy, and speech therapy) may help you recover.
Can these tumors be prevented from forming?
There is no known way to prevent these tumors from forming. However, your doctor may recommend genetic testing if you:
- If anyone in your family has a history of developing neurofibromas.
- If you have multiple nerve sheath tumors (this could be a sign of the condition neurofibromatosis).
What should we think about these nuts going forward? (Outlook)
Most neurofibromas are noncancerous . They can be cured with surgery and rarely come back. However, if your tumor cannot be completely removed with surgery, you will need to continue to be under medical supervision.
About 3 out of 4 people who receive treatment for vestibular schwannoma are able to retain their hearing .
The risk of a nerve sheath tumor becoming cancerous is very low. The highest risk is for people with the condition (NF1) who develop Plexiform neurofibroma. The prognosis for malignant peripheral nerve sheath tumors (MPNST) is not as good, especially if the tumor is larger than 2 inches. Less than half of people with this condition survive five years after diagnosis.
When should you see a doctor?
If you notice a lump under your skin, or experience any symptoms that you think could be a neurofibromatosis , see a doctor immediately . Also, tell your doctor if anyone in your family has had these types of tumors.
Final Take-Home Message
Remember, most neuroendocrine tumors are benign, noncancerous growths that grow slowly . If you have no symptoms, your doctor may decide to just observe you. If a tumor needs to be removed, surgery is usually very successful. It is very rare for complications (such as not being able to completely remove the tumor, nerve damage) to occur, or for a tumor to turn cancerous. Your doctor will help you develop a treatment plan and a schedule for future tests to help you manage your condition. So don't be afraid to talk to your doctor about anything.
` nerve sheath tumors, schwannoma, neurofibroma, neurofibromatosis, nerve tumors, cancer, genetic diseases, nerve sheath tumors
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