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KMID : 0359020070340020083
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Korean Journal of Gastrointestinal Endoscopy
2007 Volume.34 No. 2 p.83 ~ p.87
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A Case of Early Gastric Adenocarcinoma and Intraabdominal Schwannoma in a Patient with Neurofibromatosis Type 1
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Im Dong-Han
Whang Hyun-Chul
Oh Joon-Seok
Kim Hyun-Joo
Lee Hwa-Mock
An Jin-Kwang
Kim Kwang-Jin
Cheong Jong-Yun
Park Won-Il
Yang Ung-Suk
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Abstract
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Neurofibromatosis is an autosomal dominant hereditary disorder with an overall incidence of one in 3,000~4,000, and type 1 (Von Recklinghausen’s neurofibromatosis) characterized by the presence of multiple cutaneous neurofibromas, axillary and groin freckling, and cafe- au-lait spot. The neurofibromatosis type 1 gene is a tumor suppressor gene. Patients with the neurofibromatosis type 1 are at increased risk of developing nervous system neoplasm, including plexiform neurofibromas, optic gliomas, ependymomas, meningiomas, astrocytomas, and pheochromocytomas. Neurofibromas may undergo secondary malignant degeneration and sarcomatous changes. Patients with neurofibromatosis type 1 show a high incidence of Wilm’s tumor, rhabdomyosarcoma, nonlymphocytic leukemia, and pheochromocytoma but the gastrointestinal involvement appears to be relatively rare and usually consists of neurofibroma, ganglioneuroma, and leiomyoma. We have identified a case of early gastric adenocarcinoma and intraabdominal schwannoma in a 65-year-old man afflicted with neurofibromatosis type 1.
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KEYWORD
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Neurofibromatosis type 1, Adenocarcinoma, Schwannoma
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