黑斑息肉综合征六例临床分析

Clinical analysis of Peutz-Jeghers syndrome:a report of 6 cases

目的探讨黑斑息肉综合征(Peutz-Jegherssyndrome,PJS)的诊断方法和合理的治疗方案。方法对我院收治的6例PJS患者的临床资料进行分析。结果6例均以反复发作的腹痛起病,以肠套叠和息肉出血入院。4例患者的父亲,3例患者的祖父和1例患者的母亲患有PJS,3例患者有癌症家族史。病例4和病例5由于肠道内息肉复发引起肠套叠或反复腹痛而多次接收手术治疗。病例1和4的息肉同时伴有腺瘤样改变,病例2息肉同时伴有胃癌。治疗主要为息肉切除和部分小肠、结肠切除。结论PJS患者有明显的癌症家族史和息肉的高复发率;手术是治疗的主要手段;对PJS患者的胃肠道和其他有可能恶变的脏器应进行随访。

Objectives To investigate the diagnostic methods and reasonable treatment of Peutz- Jeghers syndrome(PJS). Methods Clinical data of six patients with PJS were reviewed. Results Repeated abdominal pain,intussusception and intestinal polyp with bleeding were main manifestations. Four patients’ father,three patients’ grandfather and one patient’ s mother were diagnosed with PJS. Three patients had family history of cancer. Case 4 and case 5 underwent laparotomy for many times because of intussusceptions caused by polyps or recurrent abdominal pain. Case 1 and case 4 had polyps synchronous with adenoma,and case 2 had polyp with gastric cancer. Main treatment included polyp resection and partial small intestinal and colon resection. Conclusions Patients with PJS have family history of cancer and a high incidence of polyp recurrence of small intestine. Surgical intervention is the first choice regimen. Surveillance should be emphasized on gastrointestinal tract and other potential malignant organs in PJS patients.