Benign tumors can grow along nerves anywhere in the body, causing complications such as pain, mobility issues, and psychosocial issues.
Neurofibromatosis 1-associated plexiform neurofibromas (NF1-PN) are benign tumors that develop along peripheral nerves throughout the body. They are typically present at birth or develop in early childhood and may grow as a person gets older.
Depending on where these tumors form, they can cause a variety of complications that can affect a person’s health and well-being. This article will explore the potential complications of NF1-PN and provide steps to help people manage or treat these concerns.
Pain is one of the most common symptoms that people with NF1-PN report. A
Significant pain is uncommon, but pain may become worse or more frequent over time. Worsening, severe pain may indicate disease progression and malignant transformation, which occurs in
Each person may experience pain caused by NF1-PN differently. Some people may have episodic pain, whereas others may have chronic, long lasting pain. Others may experience both chronic and episodic pain in different parts of the body. Some may have localized pain, while others may feel more diffuse, generalized pain.
The medical community does not yet fully understand the sources of pain from NF1-PN. Pain may result from a variety of factors, such as pressure on nerves or bones and the generation of local and systemic inflammation.
NF1-PN may also cause motor dysfunction, or difficulties with movement. The 2018 review found that about
As with pain, motor dysfunction can be progressive, increasing in frequency or severity over time. Information on the causes of motor dysfunction with NF1-PN is limited, but according to the same review, some data suggest that it is more common in people with larger tumors.
Severe motor dysfunction that affects mobility or self-care abilities is uncommon, especially in younger people. However, motor dysfunction may contribute to motor delays during development.
Children with NF1-PN should have regular assessments with a specialist who is familiar with NF1, and adaptive plans may be necessary to help with educational and social access.
People with NF1-PN may experience a wide variety of psychosocial complications that can negatively affect both physical and emotional health.
In a small 2023 survey of 35 adults with NF1-PN,
According to a
Children with NF1-PN may also have a
Other complications that may develop with NF1-PN typically depend on the particular nerves or body structures the condition is affecting. Possible complications include:
- airway or breathing problems
- bowel and bladder dysfunction
- vision issues, which may contribute to learning disabilities
- cardiovascular complications
- spinal cord compression, which can cause other neurological problems
- changes in growth, such as short stature and larger-than-average head size
- increased risk of certain types of cancer, including breast cancer, gastrointestinal tumors, and gliomas
Management of NF1-PN often requires a multidisciplinary team of experts who specialize in the various body systems the condition affects. These healthcare professionals can help with both monitoring and treatment as new or worsening symptoms develop.
In many cases, people can manage complications of NF1-PN with symptom-specific care. For instance, people commonly use pain medication to help manage pain from NF1-PN. In a
PN-specific medication can also help manage PN symptoms. In a small
Cognitive behavioral therapy (CBT) may also be helpful for managing symptoms of NF1-PN, including pain, functional limitations, and emotional health challenges. Healthcare professionals may also recommend other symptom-specific medical approaches, such as continuous positive airway pressure (CPAP) for sleep apnea or physical therapy for mobility problems.
In some cases, healthcare professionals may recommend surgical removal of tumors to help relieve symptoms of NF1-PN. However, results from a large study involving more than 1,000 people with NF1-PN suggest that about one-third of tumors are inoperable.
A team of experts who have experience treating NF1-PN and its associated complications should closely monitor anyone who has the condition. Careful monitoring can help healthcare professionals detect changes in the frequency or intensity of symptoms that may be cause for concern or may require new or increased intervention.
Because NF1-PN tumors may develop or grow over time, people with NF1-PN should report any changes in symptoms to their healthcare team in a timely manner.
Although NF1-PN tumors are often benign, they can affect many organ systems and lead to a variety of complications. These complications may change or worsen over the course of the disease. Regular monitoring and timely communication with a healthcare team can help people address any disease-related complications as quickly as possible.
Managing NF1-PN complications may involve symptom-specific care or treatments that address the underlying cause (the tumors). Disease-specific treatments for NF1-PN may include medication and surgical removal of problematic tumors. A person and their healthcare team will typically make treatment decisions on an individual basis.