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Intestinal Research 2004;2(1):31-35.
Published online April 16, 2004.
Two Cases of Desmoid Tumor After the Total Colectomy in FAP Patients
Bo Young Hwang, Hyo Jong Kim, Jae Young Chang, Nam Hoon Kim, Sang Kil Lee, Kwnag Ro Joo, Seok Ho Dong, Joung Il Lee, Byung Ho Kim, Young Woon Chang, Rin Chang
Department of Internal Medicine, Kyung Hee University College of Medicine, Seoul, Korea
가족성선종성용종증 수술 후 발생한 유건종 2예
황보영, 김효종, 장재영, 김남훈, 이상길, 주광로, 동석호, 김병호, 장영운, 이정일, 장린
경희대학교 의과대학 내과학교실, 소화기병 연구소
Abstract
Desmoid tumors (DT) are rare and slow-growing tumor that consist of proliferation of well-differentiated fibroblast. Although the typical characteristics of malignant tumors, such as distant metastasis, are absent, the tumor are locally aggressive and grow into neighbouring structures and have a high propensity for recurrence after surgical resection. Surgical trauma, genetic predisposition and hormonal factors are considered to be correlated with the development and growth of DT. The prevalence of desmoid tumors in patients with familial adenomatous polyposis (FAP) is 7-12%. The lifetime risk of developing desmoid tumors is about 20%. Inpatients with FAP, desmoids often appear after total colectomy as descrete masses in surgical scars of the abdominal wall or as infiltrating fibroblast sheets within the abdominal mesentery or retroperitoneum. Intra-abdominal or mesenteric desmoids pose a serious management problem because of their propensity to surroud and compress major blood vessels and viscera. Next to colorectal cancer, desmoid tumors are the most frequent cause of death in FAP. There are no standard medical approaches. Recently we experienced two cases of desmoid tumor associated with FAP after total colectomy. One case treated with non steroidal anti-inflammatory drug (NSAID) and tamoxifen, the other with combination chemotherpy. We report comparision of two cases with review of the literatures. (Intestinal Research 2004;2:31-35)
Key Words: Desmoid tumor, FAP
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