Korean J Gastrointest Endosc.  2007 Feb;34(2):83-87.

A Case of Early Gastric Adenocarcinoma and Intraabdominal Schwannoma in a Patient with Neurofibromatosis Type I

• Im DH
• Whang HC
• Oh JS
• Kim HJ
• Lee HM
• An JK
• Kim KJ
• Cheong JY
• Park WI
• Yang US

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.

Keyword

Neurofibromatosis type I; Adenocarcinoma; Schwannoma

MeSH Terms

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