Desmoid Tumor in Familial Adenomatous Polyposis (FAP).
10.3393/jksc.2008.24.1.20
- Author:
Dae Dong KIM
1
;
Chang Sik YU
;
Dong Hyun HONG
;
Sang Hun JUNG
;
Pyong Wha CHOI
;
In Ja PARK
;
Hee Cheol KIM
;
Jin Cheon KIM
Author Information
1. Colorectal Clinic, Department of Surgery, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea. csyu@amc.seoul.kr
- Publication Type:Original Article
- Keywords:
Familial adenomatous polyposis;
Desmoid tumor
- MeSH:
Abdominal Wall;
Adenomatous Polyposis Coli;
Fibromatosis, Aggressive;
Humans;
Incidence;
Postoperative Period;
Recurrence
- From:Journal of the Korean Society of Coloproctology
2008;24(1):20-26
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: This research was conducted to assess the incidence, clinical characteristics, and treatment outcomes for desmoid tumors in patients with familial adenomatous polyposis (FAP). METHODS: At Medical Center, we recruited 47 patients who had been diagnosed as having intraabdominal or abdominal wall desmoid tumor between Aug. 1995 and Dec. 2005. We compared FAP-associated desmoid tumors with non-FAP-associated desmoid tumors according to clinical characteristics and treatment outcomes. RESULTS: Desmoid tumors developed 12/46 (26.1%) in FAP, 1/14 (7.1%) in attenuated FAP and 34 in non-FAP associated. Unlike non-FAP-associated desmoid tumors, the occurrence of FAP-associated desmoid tumors in tended to be higher in the earlier age groups (< or =40 yrs, 92.3% vs 67.6%, P=0.082) and no sexual predominancy was observed (male:female ratio of 1.2:1 vs a tumor ratio 1:3.9, P=0.033). Intraabdominal-type desmoid tumors associated for the majority of FAP-associated desmoid tumors (92.3% vs 38.2%, P=0.002), and 70% of the desmoid tumors occurred within 3 years after total proctocolectomy. In the treatment of FAP-associated intraabdominal desmoid tumors, surgery was performed in 7 cases (58.3%), and complete resections were done in only 3 cases (25%), with one recurrence. In non-FAP-associated desmoid tumors, complete resection was possible in 10 cases (76.9%), and there was no recurrence (P=0.036). The medical treatment for unresectable or incompletely resectable cases in cases of non-FAP-associated desmoid tumor was good, but for FAP-associated desmoid tumors, the effectiveness was not good, and further investigation was needed. CONCLUSIONS: Intraabdominal desmoid tumors in FAP patients occurred frequently in the early (< or =3 yrs) postoperative period, and the treatment, outcome including surgery and medication, outcome was not good in patients with FAP-associated desmoid tumors.
