Neurofibroma
| Classification according to ICD-10 | |
|---|---|
| D36 | Benign neoplasm in other and unspecified locations |
| D36.1 | Peripheral nerves and autonomic nervous system |
| D48 | New formation of unsafe or unknown behavior at other and unspecified locations |
| D48.2 | Peripheral nerves and autonomic nervous system |
| Q85.0 |
Neurofibromatosis (not malignant) - von Recklinghausen disease |
| ICD-10 online (WHO version 2019) | |
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Neurofibromas are nerve tumors that form easily demarcated structures within the nerve or diffuse structures outside the nerve. They consist of neoplastic Schwann cells , fibroblasts and perineural cells , which are embedded in a matrix of collagen fibers . They are classified as level I on the WHO scale from I-IV. They differ from schwannoma in that they contain a higher amount of connective tissue and in that they cannot be surgically separated from the nerves and these usually have to be sacrificed.
They can appear as solitary nodules practically anywhere where nerve tissue is present. Most often they appear on the skin . Multiple occurrences are observed in particular in neurofibromatosis type 1 , which is due to a mutation in the NF1 gene . The plexiform variant occurs primarily on larger nerves and there is a risk of degeneration into malignant peripheral nerve sheath tumor (MPNST). The myxoid neurofibroma is typical of the NAME syndrome .
therapy
Neurofibromas of the skin can be removed for cosmetic reasons, also because patients with pronounced changes can become socially isolated. The therapy with the carbon dioxide laser, which can remove a large number of fibroids in one treatment session, has proven itself here. However, a scar-free removal is not possible even with laser treatment. The surgical removal of fibroids in the central nervous system and on peripheral nerves is associated with the risk of functional failure.
literature
- Jean-Christophe Drappier et al .: Medical management of neurofibromatosis 1: a cross-sectional study of 383 patients. In: J Am Acad Dermatol. 49 (3), Sep 2003, pp. 440-444. PMID 12963907 .
Web links
Individual evidence
- ↑ orphanet ; Retrieved on Apr 25, 2014.
- ↑ DJ Atherton, DW Pitcher, RS Wells, DM MacDonald: A syndrome of various cutaneous pigmented lesions, myxoid neurofibromata and atrial myxoma: the NAME syndrome. In: Br J Dermatol . 103 (4), Oct 1980, pp. 421-429, PubMed PMID 7437308 .
