Neurofibromatosis

Neurofibromatoses are a group of genetically heterogeneous diseases which include the independent diseases type 1 (NF1), type 2 (NF2), and schwannomatosis. At over 90% of all neurofibromatoses, NF1 is the most common of these diseases. NF2 is much more rare. From an aesthetic/plastic surgery perspective, surgical treatment focuses on the removal of schwannomas (benign, usually slow growing tumors), which stand out because of their location and/or size or cause functional complaints. This treatment option applies also to schwannomatosis.
 
The Clinic of Oral and Maxillofacial Surgery has been offering surgical treatment of neurofibromatosis type 1 for 20 years, under special consideration of plastic and reconstructive techniques. NF1 is characterized by a great number of symptoms and findings which require interdisciplinary care. With its many specialty departments, the UKE is optimally equipped for this purpose.
 
Nerve sheath tumors in NF1
A major problem for NF1 patients is the peripheral nerve sheath tumors, i.e. neurofibromas, occurring in varying amounts. Most neurofibromas are benign tumors that frequently grow in the skin and may have a disfiguring effect. Removal of these tumors is usually desired for aesthetic reasons. In addition, plexiform neurofibromas are expected in approximately 30% of patients. These tumors are usually much bigger, encompassing larger anatomical units and potentially affecting entire body segments. Plexiform neurofibromas are tumors that may be associated with substantial functional impairment and are considered cancer precursors (precancer).

The length of hospital stay varies depending on the type and spread of the tumor. After removal of skin tumors, patients can expect to be released into outpatient care after 3-4 days. Inpatient care of patients with plexiform neurofibromas can increase to 2 weeks or more in order to be able to quickly initiate any necessary post-operative procedures.

After the operation, the patient is transferred to one of our wards. For complex procedures that require close monitoring of the wound or intensive care of the patient for general health reasons, an intermediate care unit and an intensive care unit are available. A pediatric intensive care unit is accessible to children with NF1.

After the surgical treatment, follow-up of the general condition, and assessment of the wound healing process, patients are discharged to outpatient follow-up in their home countries; follow-up care takes place in close consultation with treating physicians in the patients‘ home countries. Upon request, initial follow-up may occur in the outpatient clinic of the UKE.

Prognosis
NF1 is a chronic progressive disease for which no effective drug treatment exists. Surgical treatment may reduce the number and spread of neurofibromas but does not influence the genetic basis of the disease or the tumor biology. Surgical treatment of plexiform neurofibromas is usually limited to the reduction of the size of extensive tumors, frequently in several steps. Complete removal of small plexiform neurofibromas is successful mainly in childhood. Scars are to be expected in all cases. Based on our longterm experience, however, these scars heal in an aesthetically favorable manner despite difficult healing conditions caused by the congenital connective tissue disorder.

Please direct your treatment request to the UKE International Office.

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University Medical Center
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