Overview
Malignant peripheral nerve sheath tumors are rare cancers that originate in the lining of the nerves, specifically in the peripheral nerves that run from the spinal cord into the body. They used to be called neurofibrosarcomas. These tumors can occur anywhere in the body, most commonly in the deep tissue of the arms, legs, and trunk. They typically cause pain and weakness in the affected area and may present as a growing lump or mass. Surgery is the primary treatment option, and sometimes radiation therapy and chemotherapy may also be used.
Understanding the disease
Symptoms
The symptoms of malignant peripheral nerve sheath tumors include pain where the tumor is growing, weakness when trying to move the body part that has the tumor, and a growing lump of tissue under the skin. These symptoms can worsen quickly.
Risk Factors
Risk factors for developing malignant peripheral nerve sheath tumors include radiation therapy for cancer, noncancerous nerve tumors, and a condition that runs in families called neurofibromatosis 1, which increases the risk of tumors in the nerves.
Development
Malignant peripheral nerve sheath tumors develop when cells in the lining around a nerve experience changes in their DNA, leading to uncontrolled growth and the formation of tumors. These tumors can grow into and kill healthy body tissue, and over time, the cells can spread to other parts of the body. The various types of malignant peripheral nerve sheath tumors can arise from noncancerous nerve tumors or occur in individuals with a condition that increases the risk of tumors in the nerves, such as neurofibromatosis 1. Radiation therapy for cancer can also increase the risk of developing these tumors, with malignant peripheral nerve sheath tumors occurring in the treated area 10 to 20 years after therapy.
Assessment and Diagnosis
Triage
You should see a doctor if you have any symptoms of malignant peripheral nerve sheath tumors, such as pain where the tumor is growing, weakness when trying to move the body part that has the tumor, or a growing lump of tissue under the skin. It is important to seek medical attention, as malignant peripheral nerve sheath tumors are rare, and your healthcare provider may initially look for more common causes of your symptoms.
Diagnosis
To diagnose malignant peripheral nerve sheath tumors, a healthcare provider will typically perform a neurological exam, imaging tests like MRI or CT scans, and a biopsy. Imaging tests help determine the size of the tumor and whether it has spread to other parts of the body. A biopsy involves removing a sample of tissue for laboratory testing to determine if the tumor is cancerous.
Management and Treatment
Complications
Potential complications of malignant peripheral nerve sheath tumors may include:
- Pain: Malignant peripheral nerve sheath tumors can cause pain, particularly in the areas where they occur. The pain might worsen over time.
- Weakness: Due to the pressure and invasion of healthy nerve tissue, these tumors can cause weakness in the affected limbs, making it difficult for the individual to move or use those body parts.
- Loss of function: As the tumor grows and spreads, it may lead to a loss of function in the affected area, such as difficulty in walking or performing daily tasks.
- Numbness: Malignant peripheral nerve sheath tumors can cause numbness or a loss of sensation in the affected areas.
- Spread to other parts of the body: These tumors can metastasize and spread to other parts of the body, such as the lungs or bones, which can further complicate the situation and reduce the chances of successful treatment. It is essential to seek medical attention if you experience any symptoms related to malignant peripheral nerve sheath tumors, as early detection and treatment can potentially improve outcomes and minimize complications.
Home Remedies
It is important to consult a healthcare provider for proper diagnosis and treatment of malignant peripheral nerve sheath tumors. Surgery and other medical treatments are typically recommended for managing these types of tumors. Home remedies may not be effective in addressing the underlying cause or potential complications of this condition.
Treatment
The usual treatment for malignant peripheral nerve sheath tumors is surgery. In some cases, treatment may also include radiation therapy and chemotherapy. These tumors can cause pain and weakness, and removing the tumor is often necessary to alleviate these symptoms. However, it's important to note that malignant peripheral nerve sheath tumors are rare cancers that start in the lining of the nerves.
Preparing for medical consultation
To prepare for an appointment for malignant peripheral nerve sheath tumors, you can:
- Make a list of your symptoms, including when they began.
- Prepare a list of all medications, vitamins, and supplements you are taking, including doses.
- Compile a list of questions to ask your healthcare provider, including questions about the cause of your symptoms, necessary tests, and the next steps for diagnosis and treatment.
- If you are seeing a specialist, prepare questions about their recommendations for treatment, the potential side effects, and the outlook for your condition.
- Bring a family member or friend for support to help remember the information provided during the appointment.
- Research peripheral nerve tumors to gain more knowledge about the condition and potential treatments. Consult with a counselor or social worker if needed.
- Maintain a strong support system through your family, friends, or support groups. Remember to ask any additional questions you may have during the appointment. Good preparation will help you make informed decisions about your treatment and care.