NF1-PN: Management and Risks of Neurofibromatosis Tumors

NF1-PN plexiform neurofibromas

Hello Dr.
Hu: My son is 12 years old now.
When he was 8, we discovered that he had a tumor in his abdomen and liver.
Because it is a flat lesion, we have visited 7 or 8 major hospitals, but no doctor has dared to perform surgery on him.
I would like to ask if this is truly unmanageable and if we can only monitor it regularly.
Is there a possibility that it could become malignant?

Yixuan, 40~49 year old female. Ask Date: 2022/03/20

Dr. Hu Ziren reply Oncology

Pediatric oncology is a specialized field of medicine that focuses on the diagnosis and treatment of cancers in children.
This branch of medicine addresses various types of pediatric tumors, including leukemia, brain tumors, neuroblastoma, Wilms tumor, and retinoblastoma, among others.
Pediatric oncologists work closely with a multidisciplinary team to provide comprehensive care, which may include chemotherapy, radiation therapy, surgery, and supportive care

Reply Date: 2022/03/20

More Info

Neurofibromatosis type 1 (NF1) is a genetic disorder that can lead to the development of various tumors, including plexiform neurofibromas (PN), which are often found in the abdominal region and can involve the liver. Your concern about your son's condition is valid, especially considering the complexity of managing NF1-related tumors.

Plexiform neurofibromas are typically benign tumors that arise from the nerve sheath and are associated with NF1. They can be challenging to treat due to their infiltrative nature and the potential for involvement of surrounding tissues and organs. In many cases, surgical intervention is not recommended unless there are significant complications, such as pain, functional impairment, or malignancy. The fact that multiple hospitals have hesitated to perform surgery indicates that they may have assessed the risks and benefits and determined that the potential complications of surgery outweigh the benefits in your son's case.

Regular monitoring is indeed a common approach for managing plexiform neurofibromas, especially when they are asymptomatic or not causing significant issues. This typically involves periodic imaging studies, such as MRI, to assess the size and characteristics of the tumors over time. The goal is to detect any changes that might indicate growth or transformation into a malignant form, although this is relatively rare.

Regarding the risk of malignant transformation, it is important to note that while plexiform neurofibromas are generally benign, there is a small risk (estimated at around 5-15%) that they can undergo malignant transformation into a type of cancer known as malignant peripheral nerve sheath tumor (MPNST). This risk is higher in individuals with NF1 compared to the general population. Therefore, it is crucial to remain vigilant and report any new symptoms, such as increased pain, rapid growth of the tumor, or neurological deficits, to your healthcare provider.

In terms of management, a multidisciplinary approach is often beneficial. This may include consultations with pediatric oncologists, geneticists, and specialists in neurofibromatosis. Genetic counseling can provide valuable information about the hereditary nature of NF1 and its implications for your family. Additionally, engaging with support groups or organizations focused on NF1 can provide resources and connect you with others who have similar experiences.

In summary, while it may seem frustrating that surgery is not currently an option for your son's plexiform neurofibromas, regular monitoring and a comprehensive management plan are essential components of care. It is crucial to maintain open communication with your healthcare team and to stay informed about any changes in your son's condition. If you have further concerns or if new symptoms arise, do not hesitate to seek a second opinion or further evaluation from specialists experienced in managing NF1 and its associated tumors.

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