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題 名 | Familial Adenomatous Polyposis Complicated with Desmoid Tumor of the Chest Wall: A Case Report and Literature Review=家族性大腸腺樣息肉症併胸壁硬纖維瘤:病例報告 |
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作 者 | 許正義; 許競文; 金仁; | 書刊名 | 臺灣外科醫學會雜誌 |
卷 期 | 36:3 民92.05-06 |
頁 次 | 頁151-155 |
分類號 | 416.245 |
關鍵詞 | 家族性大腸腺樣息肉症; 胸壁; 硬纖維瘤; Chest wall tumor; Desmoid tumor; Familial adenomatous polyposis; |
語 文 | 英文(English) |
英文摘要 | Desmoid tumor (aggressive fibromatosis) is a benign neoplasm with a high rate of recurrence, but without metastasis, it has been reported as being one of the most common histological subtypes of chest wall soft tissue sarcoma. Risk factors for desmoid tumor, such as prior surgery, age at desmoid tumor diagnosis, pregnancy, and family history were sought. The literature review indicated that desmoid tumors are exceedingly common in familial adenomatous polyposis <FAP) where the comparative risk is 852 times that of the general population. A germ-line mutation of the adenomatous polyposis coli (APC) gene and overexpression of beta- catenin are involved in the development of the desmoid tumor in FAP. These tumors are chiefly found in the intra-abdominal cavity and abdominal wall but rarely in the chest wall. We present a case of a 21-year-old young man. He was admitted with a protruding anterior chest wall mass which he had for 5 years, He also had a history of FAP and underwent a colectomy 8 years ago. Resection of the tumor with chest wall reconstruction was performed. No adjuvant postoperative therapy was done. Twelve months after the operation, there was no evidence of recurrence. The main 'treatments which include pharmacological therapy, hormonal therapy, radiation therapy, chemotherapy have all been reported. The surgical treatment with aggressive wide excision is the main choice of therapy. Because of the high recurrence rate of desmoid tumors, we intend to follow up this patient for a long period. |
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