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目的 研究黑斑息肉病病人肿瘤风险,明确其肿瘤易感性。方法 调查、总结治疗和收集的5 个黑斑息肉病家系32 例病人和7 例散发性病人,并结合国内近10 年的文献34 篇加以分析。结果 共169 例病人,恶性肿瘤发生率为20-1 % ,其中家族性病人90 例,恶性肿瘤发生率为20 % 。病理类型以低分化粘液腺癌为多。平均确诊年龄32-2 岁。消化道癌中,错构瘤癌变5 例,腺瘤癌变2 例。结论 黑斑息肉病病人是典型的恶性肿瘤高发人群。常见肿瘤依次为大肠癌、胃癌、小肠癌、宫颈癌和卵巢癌等。恶性肿瘤发病年龄轻,分化较差。在黑斑息肉病病人中存在错构瘤- 腺瘤- 癌的肿瘤发生途径,病人的一些( 发生LKB1 杂合性缺失的) 错构瘤和腺瘤性息肉应被视为癌前病变。
Objective To study the tumor risk of patients with polyps and to clarify its tumor susceptibility. Method investigation, summarizes the treatment and collection of five families of melasma polyps 32 patients and 7 cases of sporadic patients, combined with the domestic literature in the past 10 years to analyze 34. Results A total of 169 patients, the incidence of malignant tumors was 20-1%, of which 90 cases of familial patients, the incidence of malignant tumors was 20%. Pathological types of poorly differentiated mucinous adenocarcinoma. The average age of diagnosed 32-2 years old. Gastrointestinal cancer, hamartoma carcinogenesis in 5 cases, adenoma in 2 cases. Conclusions Patient with melasma is a typical malignant tumor. Common tumors followed by colon cancer, stomach cancer, small intestine cancer, cervical cancer and ovarian cancer. The incidence of malignant tumors is young, poorly differentiated. In hamartoma patients there is a hamartoma-adenoma-cancer tumorigenic pathway in which some of the patients (with LKB1 loss of heterozygosity) hamartomas and adenomatous polyps should be considered precancerous lesions.