| Desmoid Tumor in Familial Adenomatous Polyposis (FAP). |
|
Kim, Dae Dong , Yu, Chang Sik , Hong, Dong Hyun , Jung, Sang Hun , Choi, Pyong Wha , Park, In Ja , Kim, Hee Cheol , Kim, Jin Cheon |
|
Colorectal Clinic, Department of Surgery, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea. csyu@amc.seoul.kr |
|
|
|
|
|
| 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. |
| Key Words:
Familial adenomatous polyposis; Desmoid tumor |
|
PDF Links
Full text via DOI
