黑斑息肉病6例临床分析报告

CLINICAL ANALYSIS ON 6 CASES OF PEUTZ-JEGHERS SYNDROME

目的探讨黑斑息肉病的临床、影像、病理和内镜表现和治疗对策。方法结合国内外文献复习,对6例黑斑息肉病患者的临床资料进行综合分析。结果6例均以反复发作的腹痛、不完全性肠梗阻或便血就诊,伴有皮肤粘膜黑斑,4例有家族史。胃肠钡餐、胃镜和结肠镜检查均见消化道多发性息肉,病理上为错构瘤样或增生性息肉,内镜下息肉摘(切)除和部分肠袢手术切除效果良好。结论黑斑息肉病以特殊部位的皮肤粘膜黑斑和消化道多发错构瘤样或增生性息肉为特征,以手术切除为主要治疗手段,并重视定期随访,以早期发现息肉恶变和胃肠道内外并发的恶性肿瘤。

Objective To analyze the clinical features, imaging manifestation, pathological findings, endoscopic findings and treatment regimen of Peutz - Jeghers syndrome （PJS）. Methods The overall clinical data of 6 cases with PJS were retrospectively reported and the related literature within recently years were reviewed. Results Six patients had recurrent abdominal pain,incomplete intestinal obstruction or hematochezia, together with the pigmentation of perioral skin, oral mucosa and skin in distal extremities. Four of 6 patients had obvious family history. Multipie polyps in the digestive tract were found by gastrointestinal barium meal examination, gastrescopy and eolonscopy. The pathological findings showed hamartoma - like or hyperplastie polyps. The clinical treatment of the endoscopic polypectomy and partial enterectomy were effective. Conclusion PJS is characterized by signs of pigmentation of perioral skin and oral mucesa together with mutiple hamartoma - like or hyperplastie polyps in digestive tract. Surgical intervention is the first choice regimen and the regular follow - up should be emphasized on PJS patients in order to earlier find malignant change of polyps and other complicated malignant tumors.