A Case of Early Gastric Adenocarcinoma and Intraabdominal Schwannoma in a Patient with Neurofibromatosis Type I.
- Author:
Dong Han IM
1
;
Hyun Chul WHANG
;
Joon Seok OH
;
Hyun Ju KIM
;
Hwa Mock LEE
;
Jin Kwang AN
;
Kwang Jin KIM
;
Jong Yun CHEONG
;
Won Il PARK
;
Ung Suk YANG
Author Information
1. Division of Gastroenterology, Department of Internal Medicine, Bong Seng Memorial Hospital, Busan, Korea. usyang112@yahoo.co.kr
- Publication Type:Case Report
- Keywords:
Neurofibromatosis type I;
Adenocarcinoma;
Schwannoma
- MeSH:
Adenocarcinoma*;
Aged;
Astrocytoma;
Ependymoma;
Ganglioneuroma;
Genes, Tumor Suppressor;
Groin;
Humans;
Incidence;
Leiomyoma;
Leukemia;
Meningioma;
Nervous System Neoplasms;
Neurilemmoma*;
Neurofibroma;
Neurofibroma, Plexiform;
Neurofibromatoses*;
Neurofibromatosis 1*;
Optic Nerve Glioma;
Pheochromocytoma;
Rhabdomyosarcoma;
Wilms Tumor
- From:Korean Journal of Gastrointestinal Endoscopy
2007;34(2):83-87
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
