Gardner's Syndrome A case report.
- Author:
Dong Won JUNG
1
;
Dong Yi KIM
;
Hyeong Rok KIM
;
Young Jin KIM
Author Information
1. Division of Gastroenterologic Surgery, Chonnam University Medical School, Kwangju, Korea.
- Publication Type:Case Report
- Keywords:
Gardner's Syndrome;
Familial Adenomatous Polyposis(FAP)
- MeSH:
Adenoma;
Adenomatous Polyposis Coli;
Adenomatous Polyps;
Adolescent;
Adult;
Arm;
Chromosomes, Human, Pair 5;
Colectomy;
Colon;
Diagnosis;
Epidermal Cyst;
Fibroma;
Fibromatosis, Aggressive;
Gardner Syndrome*;
Humans;
Lipoma;
Osteoma;
Rectum
- From:Journal of the Korean Society of Coloproctology
1997;13(2):291-300
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Familial adenomatous polyposis(FAP) is a hereditary disorder characterized by the development in adolescence or early adult life of multiple adenomatous polyps throughout the colon and rectum. The risk of malignant transformation is so high as to virtually 100 per cent if the patient lives long enough. Gardner's syndrome, a variant of familial adenomatous polyposis, is characterized by colorectal adenomas, multiple osteomas and variety of soft tissue lesions, including sebaceous cysts, fibromas, lipomas, and desmoid tumors. The recent study show that this group of diseases are caused by different mutations of the same gene located on the long arm of chromosome 5. All family members at risk of inheriting this gene need frequent surveillance of the colon. This should start between the ages of 8 and 12 years and should be continued at regular intervals. Prompt colectomy is indicated at the time the diagnosis is made. We experienced a case of Gardner's syndrome, which had typical extracolonic manifestations. Herein, we report this case with a review of the literature.
