Definition
Malignant peripheral nerve sheath tumors, or also neurofibrosarcomas, are kind of sarcomas that originate in the protective line of the nerves that spread from the spinal cord into the body. They can appear anywhere in the body, but most common cases of such tumors are found in deep tissue of the arms, legs and trunk. They cause pain and weakness and sometimes a lump as well. These tumors are more frequent among people that have hereditary predisposition of developing nerve tumors and people that have already experienced radiation therapy for cancer. However, many people that suffer from malignant peripheral nerve sheath tumors don not have any risk factors. These kinds of tumors are treated only with surgery, followed by chemotherapy and radiation therapy in some cases.
Symptoms
The symptoms may include:
- Neurological symptoms
- Soreness in the area where the tumor is localized
- Swelling in the arms or legs (usually painless)
- Problems moving the affected extremities
- Pain
- Discomfort
- Numbness
- Burning
- Dizziness
- Loss of balance
Causes
It is believed that neurofibrosarcoma can be genetic as they have been discovered within families. The p53 is a tumor suppressor gene. It is believed that the p53 genome on 17p in patients with neurofibrosarcoma is mutated, which increases the probability of cancer development. People with inactivated p53 gene do not have the normal p53 gene that regulates cell growth and inhibit any uncontrollable cell growth in healthy population, so they are even more susceptible to tumors. However, the exact cause is still unknown.
Treatment
Treatment options for malignant peripheral nerve sheath tumors are surgery, radiation therapy and chemotherapy.
Surgery is usually the most effective treatment, which focuses on completely removing the tumor as well as treating local recurrence of tumors and distant metastases.
Radiation therapy is usually an integral part of the treatments for almost every soft tissue sarcomas. It can be prescribed pre-operatively or post-operatively. In combination with the surgical excision, radiation therapy has been quite effective.
Chemotherapy can also be pre-operative or post-operative treatment. As it carries side-effects the decision to treat the tumor with chemotherapy should be carefully discussed taking in consideration the individual patient’s preferences. The benefit from the chemotherapy is possible shrinking of the tumor. Usually, this kind of treatment is recommended when the tumor is less than 8 cm in diameter and when the patients are not older than 65 years with good cardiac function.
