黑斑息肉病的临床特点和治疗策略  

Clinical Features and Management of Peutz-Jeghers Syndrome

作　　者：杨斌 赵博[2] 王振军[2] 

机构地区：[1]河南省新乡市第二人民医院外科,453000 [2]北京医科大学第一医院外科,100034

出　　处：《大肠肛门病外科杂志》2004年第4期271-273,共3页Journal of Coloproctological Surgery

摘　　要：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 investigate and analyze the clinical feature and management of Chinese Peutz-Jeghers patients.Methods:The diagnosis and treatment of 165 patients' data collected from our hospital and Chinese literature in the past 10 years was analyzed.Results:Hamartomatous polyps of the gastrointestinal tract and freckling of the lips, buccal mucosa were the two main features,and abdominal pain and gastrointestinal bleeding were the main presentations, with 58.7% patients receiving operation aiming to relieve acute intestinal obstructions. 33 patients (20%) developed malignancy. Cancers developed at early age and tend to be low differentiation.Conclusion:Hamartomatous polyps of the gastrointestinal tract and freckling of the lips, buccal mucosa were the two main features.Abdominal pain and gastrointestinal bleeding were the main presentations.patients with Peutz-Jeghers patients' syndrome predispose to neoplasms of multiple organs. Early treatment of the polyps and active cancer screening is very important.

关 键 词：黑斑息肉病 临床特点 恶性肿瘤 常见 治疗策略 小肠癌 消化道出血 宫颈癌 胃癌 病人 

分 类 号：R730.5[医药卫生—肿瘤] R730.4[医药卫生—临床医学]