Erschienen in:
01.08.2014 | Scientific Letter
Labial Neurofibroma as the Presenting Feature of Genital and Orbitotemporal Neurofibromatosis
verfasst von:
Deep Dutta, Ajitesh Roy, Satinath Mukhopadhyay, Subhankar Chowdhury
Erschienen in:
Indian Journal of Pediatrics
|
Ausgabe 8/2014
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Excerpt
To the Editor: Neurofibromatosis (NF) type-1 is an autosomal dominant neurocutaneous disorder (17q11.2 defect; 1:3,000–4,000 births), characterized by peripheral nerve sheath tumors (neurofibromas), café-au-lait spots, intertriginous freckles and Lisch nodules [
1]. Genital NF is extremely rare, clitoromegaly being the most common type, and is often confused with hyperandrogenic disorders resulting in unnecessary evaluations [
1,
2]. Neurofibromas involving the orbital, temporal and facial regions are known as orbitotemporal neurofibromatosis (OTNF) and are also rare (<1 %) [
3]. …