Neurofibromatoses are a group of three genetically distinct disorders that cause tumors to grow in the nervous system. Tumors begin in the supporting cells that make up the nerve and the myelin sheath (the thin membrane that envelops and protects the nerves), rather than the cells that actually transmit information. The type of tumor that develops depends on the type of supporting cells involved. Scientists have classified the disorders as neurofibromatosis type 1 (NF1, also called von Recklinghaus disease), neurofibromatosis type 2 (NF2), and a type that was once considered to be a variation of NF2 but is now called schwannomatosis. An estimated 100,000 Americans have a neurofibromatosis disorder, which occurs in both sexes and in all races and ethnic groups.
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Surgery for Neurofibromatosis:
Surgery to remove neurofibromatosis tumors is usually performed after a full medical evaluation and history of the patient is obtained, and the NF tumor(s) have been determined appropriate for surgical removal. A skilled Plastic and Reconstruction Surgeon who has expertise in the removal of tumors and other skin/body invasive disorders, as well as knowledge of the proper closing of wounds, including flap procedures is best.
Resection (the surgical removal) of painful or disfiguring tumors is normally done under anesthesia and depending upon the procedure/patient may be performed in either an outpatient surgical center or a hospital. Neurofibromas located on parts of the body that do not include the brain, spine or directly on peripheral nerves are removed by incisions made near the tumor via a scalpel and may be done in conjunction with a process called electrodessication. Proper closure of the wound is important regarding healing as well as regarding appearance of the treatment area after healing. Therefore, a skilled plastic surgeon is necessary for the best outcome.
Electrodessication is a tool used to treat clusters of tumors (neurofibromas). This procedure allows more tumors to be removed during one surgical procedure, as opposed to individual removal of each tumor separately using only a scalpel.
Electrodessication involves the use of electrical current to desiccate or dry out and kill the neurofibroma tissue. A thin pointed knife runs a current through the neurofibroma, drying it out and killing it.
The electrodesiccation procedure is normally performed in an outpatient surgical setting and usually takes 1-3 hours depending on the number of tumors to be removed. A local anesthetic can be used when removing small clusters of tumors, however, the use of general anesthetic is necessary for tumor removal over a larger area of the body. After surgery, the patient generally goes home within one or two hours.
Electrodessication can be used alongside the removal of larger individual tumors via scalpel which allows for complete coverage of the tumor areas including tumors of various sizes.
Tumors of the Brian, Spine, and directly on Peripheral Nerves
A Neurosurgeon normally operates on benign and malignant tumors located in the brain, spine, and directly on peripheral nerves (if surgery is deemed appropriate and safe).
Benign tumors can be removed for relief of pain, improvement in appearance, and in specific cases, to improve neurologic function.
Malignant (cancerous) tumors often need to be removed in conjunction with a more comprehensive treatment strategy. These tumors may need to be evaluated and treatment options discussed with multiple physicians prior to surgery.
At times, biopsies may be surgically performed initially to evaluate tumors in order to determine the best treatment option.
The need for surgery can be highly specific, depending on the individual patient. Many neurofibromatosis patients do not need surgery, and some need multiple surgeries. Seeking a surgical consult is the best way to determine if you are a candidate for surgery to remove NF tumor(s). Ask questions at your consult and learn more about the options available to you, including all risks involved with surgery.