von Recklinghausen's Disease에 병발한 위장관종양 2예 보고
Gastrointestinal Tumors Associated with von Recklinghausen's Neurofibromatosis -A report of two cases-
소속 상세정보
박미자/Mee Ja Park
김혜연/원남희/김인선/이갑로/백승룡/Hye Yeon Kim/Nam Hee Won/Insun Kim/Kap No Lee/Seung Yong Paik
KMID : 0357919850190030345
Abstract
Neurofibromatosis, first clearly described by von Recklinghausen in 1882, is a
dominantly inherited mesodermal and ectodermal dysplasia with a broad spectrum of
clinical findings.
Most common is the classical neurofibromatosis, which has three major features: (1)
multiple neural tumors dispersed anywhere on or in the body; (2) numerous pigmented
skin lesions, some of which are "cafe au lait" spots; and (3) pigmented iris hamartomas
also called Lisch nodules. Other lesions sometimes seen in patients with von
Recklinghausen's disease include congenital malformations of various types, vascular
lesions, neurilemoms meningioma and other intracranial neoplasms, pheochromocrtoma,
medullary carcinoma of thyroid gland, neuroblastoma, ganglioneuroma and Wilms' tumor.
Approximately 10% of the patients with neurofibromatosis have the gastrointestinal
tract tumors as neurofibroma, ganglioneuroma and leiomyoma. Some of them show
evidence of malignancy.
Increased activity of the protein "nerve growth factor" in the sera of the patients with
disseminated neurofibromatosis has been reported. We would like to report here two
cases of von Recklinghausen's neurofibromatosis with involvement of the gastrointestinal
tract.
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