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KMID : 0988920040020010031
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Intestinal Research
2004 Volume.2 No. 1 p.31 ~ p.35
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Two Cases of Desmoid Tumor After the Total Colectomy in FAP Patients
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Hwangbo Young
Kim Hyo-Jong
Jang Jae-Young
Kim Nam-Hoon
Lee Sang-Gil
Joo Kwang-Ro
Dong Seok-Ho
Kim Byung-Ho
Chang Young-Woon
Lee Joung-Il
Chang Rin
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Abstract
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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.
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KEYWORD
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Desmoid tumor, FAP
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