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Bilateral segmental neurofibromatosis with gastric carcinoma
Author(s) -
Kajimoto A.,
Oiso N.,
Fukai K.,
Ishii M.
Publication year - 2007
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.2006.02247.x
Subject(s) - medicine , neurofibromatosis , gastric carcinoma , pathology , cancer
Summary Neurofibromatosis type 1 (NF1) is an autosomal‐dominant disorder characterized by café‐au‐lait spots, neurofibromas, intertriginous freckles, and Lisch nodules in the iris. Segmental neurofibromatosis is fundamentally a mosaic variant of NF1. A 66‐year‐old man visited us with bilateral segmental neurofibromatosis (SN) on the torso. He had multiple neurofibromas on the back bilaterally and on the right abdomen, and partial unilateral lentiginosis on the left abdomen and left back. The patient had noticed the development of papules and nodules on the right abdomen and bilaterally on the back 6 years previously, and had been diagnosed with early gastric carcinoma 5 years previously. To our knowledge, this is only the third reported case of SN associated with internal malignant tumours.