Neurofibromatosis is a set of complex disorders in which tumors, mostly benign (noncancerous), grow from nerve tissue. Because these disorders can affect many parts of the body, comprehensive care with access to a range of pediatric experts is especially important. At OHSU Doernbecher, we give your child:
- Coordinated care from diagnosis through treatment and monitoring.
- A full range of pediatric specialists — including neurologists, neurosurgeons, neuro-oncologists and many others — all in one place.
- Advanced expertise in treating neurofibromatosis and the diverse conditions that sometimes go along with it, including hydrocephalus, epilepsy, scoliosis, heart problems and learning disabilities.
Neurofibromatosis comes in distinct genetic types and can range from relatively mild to severe. A primary feature is development of tumors, or neurofibromas, in nerve tissue of the skin and organs.
Neurofibromatosis tends to worsen over time, but individual cases are highly variable and unpredictable. These disorders also run in families, though in about half of cases, the underlying genetic mutation occurs spontaneously.
This is the most common type, affecting as many as one in 3,000 Americans. Many children with this disorder, also called NF1, have manageable symptoms, normal intelligence and normal life expectancy.
Symptoms are frequently present at birth but can also appear in early childhood. They include:
- Neurofibromas that eventually can range from small nodules to large tumors on or under the skin or deep inside the body
- Light brown spots on the skin (often called café-au-lait spots)
- Freckles in the armpit or groin area
- Growths on the eye’s iris
- An optic nerve glioma — a tumor on the nerve that transmits visual information from the eye to the brain
- Bone abnormalities in the spine, skull or shin
About half of children with NF1 have learning disabilities. Patients tend to be shorter than average with larger-than-average heads. They are also more likely than other children to have conditions such as epilepsy, hydrocephalus, headaches, high blood pressure and defects in the heart or blood vessels. In a small portion of cases — though rarely in children — neurofibromas develop into cancer.
In a subtype called segmental or mosaic neurofibromatosis, patients have symptoms such as nodules in only one part of the body.
This type affects about one in 25,000 and is typically diagnosed in young adulthood, from ages 18 to 22.
In NF2, benign, slow-growing tumors form on nerves connected to the brain that play a role in hearing and balance. These tumors, called schwannomas because they develop in the Schwann cells that form nerve coverings, can damage hearing and cause other problems by pressing on brain tissue.
NF2 patients are at higher risk of developing certain other brain and spinal cord tumors. They can also develop cataracts at a young age as well as nerve problems such as numbness and weakness in hands or feet.
This type is very rare and typically affects adults older than 30. It’s characterized by schwannomas that occur anywhere in the body except one of the nerves affected by NF2. The tumors can cause varying degrees of pain. Some patients also have nerve problems such as numbness, tingling or weakness in fingers and toes.
- Doernbecher Children’s Hospital is nationally ranked for excellence in neurology, neurosurgery and oncology. We offer teams of specialists who work together to treat and manage your child’s neurofibromatosis and any related conditions. Our services and expertise include:
- Advanced imaging: Our neuroradiologists have expertise in the unique needs of children. They have the latest imaging technology for diagnosing and monitoring neurofibromatosis and related conditions.
- World-class neurologists and neurosurgeons: Our pediatric neurologists and neurosurgeons collaborate on treating complex conditions. Should a tumor require removal, our neurosurgeons offer advanced skills in the latest surgical techniques and imaging, plus expertise in treating related conditions such as hydrocephalus and scoliosis.
- Oncology expertise: Our team includes dedicated pediatric neuro-oncologists and nurses with deep expertise in treating brain and spinal cord tumors.
- Other services: Doernbecher Children’s Hospital also has pediatric experts for related conditions and concerns, including:
- One of the nation’s few pediatric spine clinics, with combined services in neurosurgery and orthopaedics
- Genetics services including counseling for parents concerned about passing on neurofibromatosis
- A team of pediatric heart care experts
- Oregon’s only Pediatric Epilepsy Center for children who develop a seizure disorder
- Experts in neuropsychology for assessing and treating learning deficits
- Pediatric experts in vision and hearing
- Neurofibromatosis Information Page and Neurofibromatosis Fact Sheet, National Institute of Neurological Disorders and Stroke
- Neurofibromatosis Type 1, National Organization for Rare Disorders
- Children’s Tumor Foundation, an advocacy group with information and support
- Neurofibromatosis Network, another advocacy group with information and support
- The Neuro Foundation, a London-based organization with information such as a glossary and research reports
Call 503-346-0640 to:
- Request an appointment.
- Seek a second opinion.
- Ask questions.
Parking is free for patients and their visitors.
Doernbecher Children’s Hospital
707 S.W. Campus Drive
Portland, OR 97239
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Refer a patient
- Refer your patient to OHSU Doernbecher.
- Call 503-346-0644 to seek provider-to-provider advice.