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- W146869874 abstract "Neurofibromatosis (NF) is a hereditary autosomal dominant disorder. Von Recklinghausen first described NF in 1882, which is now classified as Neurofibromatosis 1 (NF-1). NF-1 is the most commonly encountered NF which affects 1 in 4000 persons. Clinical manifestations of NF-1 include: generalized cutaneous neurofibroma, pigmented skin patches (cafe-au-lait spots), pigmented iris hamartoma (Lisch nodules), skeletal abnormally, CNS tumors, etc. The subject of this case study is a young adult male with a huge plexiform neurofibroma involving both the liver and head regions. The head tumor measured 10 x 8 x 3.5 cm3 in size, weighted approximately 180g with overlying hyperpigmented skin and an underlying congenital skull defect. A CT scan and MRI of the head and neck revealed a well defined lobulated tumor and deformed external ear. A abdominal sonogram, CT scan and MRI showed a huge plexiform neurofibroma with liver invasion. Lisch nodules and multiple cafe-au-lait spots were also found. Surgical removal of the head tumor along with an external ear reconstruction was performed. Satisfactory cosmetic results and improved hearing were achieved." @default.
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- W146869874 date "1991-12-01" @default.
- W146869874 modified "2023-09-23" @default.
- W146869874 title "Huge plexiform neurofibroma of the head and liver--case report." @default.
- W146869874 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/1795416" @default.
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