INTESTINE Vol.17 No.5(1-3)

Theme Recent advance in diagnosis and management of hereditary colorectal cancer
Title Treatment for extracolonic manifestations in familial adenomatous polyposis
Publish Date 2013/10
Author Hideyuki Ishida Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
Author Kensuke Kumamoto Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
Author Tomonori Ohsawa Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
Author Okihide Suzuki Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
Author Takeaki Matsuzawa Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
Author Tohru Ishiguro Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
Author Kouki Kuwabara Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
Author Keiichiro Ishibashi Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
Author Takeo Iwama Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
[ Summary ] In the management of extracolonic manifestations in patients with familial adenomatous polyposis, special attention must be given to desmoid tumors, duodenal polyposis and cancers (including papillary lesions) as well as papillary thyroid carcinomas. Desmoid tumors frequently occur after colorectal resection. The classification proposed by Church et al. should be considered when making treatment decisions regarding intraabdominal desmoid tumors, which can be life-threatening. Cytotoxic chemotherapy employing dacarbazine and doxorubicin may be a useful alterative treatment for patients with stage III/IV desmoid tumors. Long-term surveillance is needed for patients with duodenal polyposis according to Spigelman's classification. Surgical intervention should be considered for Spigelman’s stage IV lesions. With regard to papillary thyroid carcinomas, the risk of this cancer is extremely high in young women, and multiple lesions are common. A subtotal or hemi-thyroidectomy should be performed, if possible, since the prognosis for these patients is favorable.
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