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目的 :分析黑斑息肉病患者的临床特点 ,总结治疗和随访策略。方法 :总结具有典型表型的 16 5例患者的临床特点和诊治。结果 :在典型的 16 5名病人中 ,均具有口唇、颊黏膜黑 /褐色素沉着和肠错构瘤。临床以腹痛、消化道出血为主要表现 ,病史中因肠梗阻接受急诊手术者 97例 (5 8.7% )。发生恶性肿瘤者 33例 (2 0 % ) ,常见的恶性肿瘤依次为结肠癌 14例 ,胃癌 5例 ,小肠癌和宫颈癌各 3例 ,卵巢癌和骨肉瘤各 2例 ,胰腺癌、乳腺癌、胆管癌、肺癌各 1例 ,病理类型以低分化黏液腺癌多见 ,平均确诊年龄 32岁。结论 :口唇黏膜色素斑沉着和肠错构瘤性息肉是本病的主要表型特点 ,消化道出血、腹痛、肠梗阻为其主要表现。黑斑息肉病人是恶性肿瘤的易感人群 ,癌发生较早 ,分化较差。常见的恶性肿瘤依次为大肠癌、胃癌、小肠癌、宫颈癌和卵巢癌等 ,应积极处理肠息肉和筛查肿瘤。
Objective: To analyze the clinical features of patients with polyps and summarize the treatment and follow-up strategies. Methods: The clinical features and diagnosis and treatment of 165 patients with typical phenotypes were summarized. RESULTS: Of the 165 typical patients, all had lip and buccal mucosal black / brown pigmentation and gut hamartoma. Clinical abdominal pain, gastrointestinal bleeding as the main performance history of intestinal obstruction due to emergency surgery were 97 cases (57.7%). Malignant tumors occurred in 33 cases (20%). Common malignant tumors were colon cancer in 14 cases, gastric cancer in 5 cases, small intestine and cervical cancer in 3 cases, ovarian cancer and osteosarcoma in 2 cases, pancreatic cancer and breast cancer 1 case of cholangiocarcinoma and 1 case of lung cancer. The pathological type was more common in poorly differentiated mucinous adenocarcinoma with an average age of 32 years. Conclusion: Lips mucosa pigmentation and gut tumor are the main phenotypic features of the disease, gastrointestinal bleeding, abdominal pain, intestinal obstruction as its main performance. Black spot polyp patients are susceptible to malignant tumors, cancer occurred earlier, poorly differentiated. Common malignant tumors followed by colorectal cancer, stomach cancer, small intestine cancer, cervical cancer and ovarian cancer, etc., should actively deal with intestinal polyps and screening cancer.