Neurinoma: Causes, Symptoms & Treatment

A neurinoma is a tumor that grows from Schwann’s cells and is benign. Depending on the location of the tumor, symptoms can vary greatly from case to case; however, pain and nerve loss are particularly common. Treatment options primarily include surgical removal of the neurinoma and radiation therapy.

What is a neurinoma?

Neurinoma is a specific type of tumor and grows from Schwann’s cells. This type of glial cell grows in a spiral around the process of neurons, creating an electrically insulating layer, which increases the speed of signal transmission. Neurinomas that develop on these Schwann’s cells therefore impair the functioning of the nerve in question. Most affected individuals are between 40 and 60 years of age; however, in principle, neurinoma can develop in any age group. Because of its benign nature, it is also known as benign peripheral nerve sheath tumor (BPNST). Other names for neurinoma include: Schwannoma, Schwann cell tumor, and neurolemmoma or neurilemmoma. In very rare cases (less than one percent), neurinoma, which is benign in itself, can become a malignant neurofibrosarcoma. Arms and legs are particularly often affected by neurofibrosarcoma, while neurinoma occurs mainly in the head and neck.

Causes

Like all tumors, neurinoma results from neoplasms in the affected tissue. In neurofibromatosis type 2, the likelihood of developing a neurinoma is significantly increased. The hereditary disease leads to tumor formation in the brain, with the neoplasms predominantly clustering around the vestibulocochlear nerve. Furthermore, in many cases the spinal cord and other cranial nerves have additional tumors. Malformations of the eyes, skin changes, and abnormalities of the central nervous system as a whole are also common in neurofibromatosis type 2. In addition to the general symptoms of neurinoma and, if applicable, acoustic neuroma, this disease is accompanied by further symptoms that are due to additional tumors. Physicians distinguish between two subtypes of neurofibromatosis type 2, whereby the Feiling-Gardner type only appears after the age of 20 and is characterized by slow-growing, central tumors, whereas the onset of the disease in the Wishart type is before the age of 20 and this subtype leads to many fast-growing tumors. Less commonly, neurinomas accompany neurofibromatosis type 1; however, they also occur statistically more often there and the disease is more common overall than type 2. In addition to neurofibromas, café-au-lait spots and Lisch nodules in the iris are characteristic features of type 1 neurofibromatosis.

Symptoms, complaints, and signs

Because neurinoma can form in a variety of locations in the nervous system, the appearance of the disease is varied. Pain is often one of the symptoms; which area of the body is affected depends on the damaged nerve pathways. In some circumstances, the nerve from whose Schwann’s cells the neurinoma grows is no longer functional at all. In this context, paralysis may occur. Typically, symptoms manifest only gradually, increasing in extent and severity as the neurinoma often grows slowly. Acoustic neuroma represents a specific form of schwannoma. This is a neurinoma that grows on the vestibulocochlear nerve. The VIII. Cranial nerve innervates the inner ear and is significant for both hearing and the sense of balance. Acoustic neuroma manifests as acoustic complaints such as tinnitus and hearing problems, as well as balance problems and dizziness. Other possible symptoms include nausea, vomiting, facial numbness, facial paralysis, decreased sensitivity to pressure and touch in the external auditory canal, double vision, earaches, and headaches.

Diagnosis and disease progression

Based on the clinical picture, neurologists can often already roughly narrow down where the cause of the nervous disorder that leads to the individual complaints probably lies. An examination using magnetic resonance imaging (MRI) or other imaging techniques will visualize the tumor and may also be able to map other causes and factors. Very small neoplasms that do not yet cause any limitations cannot always be detected in this process due to the limited resolution of the imaging.

Complications

Although neurinoma is a benign and slow-growing tumor, it can cause long-term complications that require surgical removal of the tumor. When the tumor is still small, there are usually no symptoms. However, larger neurinomas often displace adjacent nerves, leading to the corresponding health complaints. If the neurinoma is located in the lumbar spine, chronic back pain that radiates to the legs occurs if the tumor is large enough, due to compression of adjacent nerves. If the tumor continues to grow, weakness of the muscle supplied by the corresponding nerve may develop. If the neurinoma is located in the cervical spine, there will be long-term chronic pain radiating to the arms and sensory disturbances in this area. A neurinoma in the spinal canal can even lead to paraplegia. There is not much space in the spinal canal, so displacement of the adjacent nerves can cause their loss of function in extreme cases. In the presence of a neurinoma in the finger or hand, compression of adjacent nerves there can cause tingling and numbness. If the auditory nerves are affected, hearing loss to deafness, dizziness and tinnitus sometimes develop. A dangerous complication is the increase of the intracranial pressure up to the congestion of the cerebrospinal fluid due to the constriction of the brain stem. In very rare cases, malignant degeneration of the tumor also occurs.

When should you see a doctor?

A doctor should be consulted as soon as the first diffuse irregularities of the organism already appear. Since neurinoma is a tumor, caution should always be exercised. Even in the case of benign tumors, a clarification must be made as soon as possible so that malignant diseases can be excluded. Swelling, pain or the development of ulcers on the body should be presented to a doctor. If there are restrictions of movement, general disturbances of mobility as well as unsteadiness of gait, a doctor is needed. Loss of function of the sensory organs, nausea, vomiting, sensory disturbances or numbness of the skin should be examined and treated. If the affected person suffers from a change in vision or hearing, there is cause for concern. Seeing double images, decreased hearing, ear pain, a feeling of pressure in the head, or headaches are warning signs from the body that should be investigated. Consultation with a physician is necessary as soon as there is paralysis in the face, disturbances in balance, and hypersensitivity to touch stimuli. Characteristic for a neurinoma is a slow increase of the existing symptoms. This leads to a gradual malaise, a decrease in performance and a general feeling of illness. If the affected person’s condition does not improve, he or she should discuss the observations with a physician so that clarification of the cause can be initiated.

Treatment and therapy

Immediate intervention is not required in every case. Neurinomas typically develop slowly, sometimes allowing the practitioner to observe the behavior of the tumor first before deciding on the type of therapy. Potential treatment risks also play a role in this consideration. As with all therapeutic approaches, the individual weighing of costs and benefits for each patient is crucial here; a blanket judgment is not possible. In a surgical procedure, doctors can remove the neurinoma to prevent further growth of the tumor and thus the spread of symptoms. However, the neurinoma grows from Schwann’s cells, which electrically insulate the affected nerve cell. Therefore, the surgeon may also have to remove part of the nerve with the tumor, which can lead to the persistence of symptoms that have already occurred. After removal, a thorough histologic examination provides further insight into the nature of the tumor. Radiation therapy is another option for treating neurinoma, using ionized radiation to affect the affected tissue. Radiation dose and duration of treatment vary depending on the patient.

Outlook and prognosis

Because neurinomas are usually benign, the prognosis is generally very good. Most tumors can be removed surgically without difficulty. This is also true for advanced or larger neurinomas. In a few cases, complications occur that can lead to secondary damage.However, these limitations are sometimes only short-lived. Hearing disorders are the most common. Extremely rarely, there are also disturbances of the vestibular nerve. A few weeks after the operation, most of those affected are usually completely healthy again. If the neurinoma has been completely removed during the operation, it usually does not recur. The life expectancy of those affected is normal. Rarely, parts of the tumor cannot be surgically removed. In these cases, it is possible for a recurrence to occur. Treatment of such a tumor capsule by radiation is often successful. In about 1% of patients, a malignant neurofibrosarcoma develops from the neurinoma. Without treatment, the prognosis is much worse. The neurinoma often continues to grow and increases intracranial pressure, which can be quite dangerous for the affected person. The earlier the tumor is treated, the better the outlook with treatment.

Prevention

Neurinomas often develop in association with type 1 and type 2 neurofibromatosis. Because both forms are genetic diseases, affected individuals can consider this fact in family planning.

Follow-up

Follow-up care for neurinoma is closely coordinated with the treating physicians, but often also with physiotherapists, speech therapists, occupational therapists, or sports therapists. It is important to see the radiologist at regular intervals, who can check whether a recurrence has occurred by taking images. Often, damage has been caused by the tumor, which continues to cause symptoms after its removal. Sensory disturbances in limbs or speech disorders, for example, are treated by the relevant therapists. These therapists often provide patients with exercises that they can continue to practice on their own at home during aftercare. Psychological support is also often very important for those affected during aftercare. The knowledge of the tumor disease and the fear of a possible recurrence can often be coped with much better through conversations with friends and family as well as the distraction of social contacts. In this context, self-help groups are often a particularly helpful component in individual follow-up care for neurinoma. People suffering from the same or a similar disease bring a special understanding to the exchange of experiences and have a whole range of practical tips for those seeking advice and help. The mental state can also be stabilized by relaxation methods such as autogenic training or progressive muscle relaxation, as well as by attending yoga classes.

What you can do yourself

A malignant neurinoma must always be treated by a doctor. The growth must be removed by surgery or radiation or chemotherapy to prevent degeneration and other complications. In the case of a benign neurinoma, therapy can be supported by a number of self-help measures and home remedies. General measures such as a change in diet and moderate exercise have proven effective. Avoiding salt and gluten can have a positive effect on the growth of the growth. Caffeine, alcohol and sugar should also be avoided, as these substances place an additional burden on the body. After a surgical intervention, rest and relaxation are the order of the day. The patient should not expose himself to unnecessary stress and must care for the surgical wound according to the doctor’s instructions. If inflammation or bleeding occurs, the doctor must be informed. Finally, it is important to go for regular cancer check-ups. If a recurrence is suspected, the patient must call in the doctor and have the unusual symptoms clarified. A neurinoma must be clarified by a doctor in any case, because self-treatment of the causes is not possible.