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Once a person is diagnosed with NF1, he or she should
heed their physician’s recommendations for follow-up
care. This often means, at a minimum, an annual physical
examination and a yearly neurological evaluation; depending
on the patient’s needs, he or she may be referred
to a specialist in neurosurgery, ophthalmology, orthopaedics,
plastic surgery, dermatology, or developmental pediatrics.
Children or adults with specific concerns may need to
be seen by a physician more frequently.
Other than the annual exam, NF1 patients should contact
their physician any time there are symptoms such as:
a rapidly growing tumor; a previously painless tumor
that becomes painful; weakness or numbness; and changes
in vision.
When an NF1-associated problem is detected, there are
often remedies that can improve the patient’s
quality of life or even be life-saving. Surgery can
be performed to correct a curved spine, remove a tumor,
or, in the case of plastic surgery, improve or restore
appearance. For some optic nerve gliomas, chemotherapy
can be effective.
Tumor removal becomes complicated when the lesion is
on or in close proximity to a nerve. It is possible
that, in the process of removing a tumor, the surgeon
could inadvertently damage a nerve. To minimize that
possibility, comprehensive NF programs, such as the
one at University Hospital, have the capability to use
intraoperative electromyography (EMG) monitoring. During
surgery, the EMG continuously records a muscle’s
electrical activity, which indicates the status of nerve
function. Any changes in electrical activity are immediately
made known to the surgeon, who can modify the procedure
to avoid causing permanent harm to the nerve.
About Neurofibrosarcomas
Neurofibrosarcomas, also known as peripheral nerve
sheath tumors, are a type of malignant, soft-tissue
tumor that can develop from plexiform neurofibromas.
While it should be emphasized that neurofibrosarcomas
are rare, occurring in only 5 percent to 10 percent
of plexiform neurofibromas, they can present complex
medical problems.
A painful or quick-growing plexiform neurofibroma can
be indicative of a neurofibrosarcoma, as can difficulty
with movement of the arms or legs. Only a physical exam,
blood tests, and imaging tests, such as X-ray, MRI,
or CT, can provide the information the doctor needs
to make a diagnosis. However, many times surgery for
a biopsy is required to make the diagnosis. If a neurofibrosarcoma
is confirmed, the tumor will be staged?an assessment
of how far it has spread.
The tumor’s stage and location and the patient’s
overall condition are important factors when it comes
to developing a treatment plan. The most common option
is surgery to remove the tumor completely and its surrounding
tissue; radiation or chemotherapy are sometimes used
in combination before or after surgery, but rarely as
a stand-alone treatment. When the limbs are involved,
surgeons will do everything they can to remove the tumor
and preserve the limb, which can involve bone grafting
or insertion of a rod. This is known as limb-salvage
surgery. When the tumor cannot be removed, it may be
necessary for the affected limb to be amputated. These
decisions are made in close consultation between the
surgeon and the oncologists and are never undertaken
without the patient’s knowledge and consent.
There is no cure for NF1, but its potential damage
can be minimized if patients take a proactive approach
and routinely seek medical evaluation and care. Catching
a problem early makes an important difference in the
quality of life for people with NF1.
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Treatment
of NF1
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