Gardner综合征之软组织肿瘤的追踪观察与研究

目的：分析研究Gardner综合征一家系12例患者的软组织肿瘤的自然演变过程与结肠息肉癌变之间的关系。方法：自1989年3月至1998年10月对一家系12例Gardner综合征患者进行了长期追踪观察，采用人体体表摄影，X线平片照像，消化道气钡双对比造影，纤维结肠镜及手术切除，10年中每年观察1-2次，重点患者2次以上。结果：12例均患有软组织肿瘤。活检证实；硬纤维瘤4例。皮脂腺囊肿2例。脂肪瘤2例。神经纤维瘤2例。混合性肿瘤2例。在结肠息肉癌变的5例患者中软组织肿瘤均有不同程度的变化。表现为突然增大，增多，表面发生溃破等。结论：软组织肿瘤的突然增大增多与结肠息肉癌变有密切关系。

Tracing Observation and Study on Soft Tissue Tumor of Gardner′s Syndrome

Objective:To study the relations between the natural development of soft tissue tumor and the malignant degeneration of colonic polyp by following up12cases with Gardner' syndrome in a family.Methods:12cases with Gardner's syndrome in a family were studied from March,1989till October,1998.The examinations consisted of body photography,plain X-ray,air-barium double contrast radiography of alimentary canal,fiber-optic colonoscopy and re-section.During the10years,the examinations were taken once or twice a year,the key cases over twice a year.Results:Soft tissue tumor was present in12cases.After biopsy,desmoid was found in4cases,sebaceous cyst in2cases,lipoma in2case,neurofibroma in2cases and mixed tumor in2cases.In five cases of the malignant degeneration of colonic polyp,soft tissue tumor varied abruptly on volume expansion,amount increasing and surface ulceration.Conclusion:The abrupt volume expansion and amount increasing of the soft tissue tumor is closely related with the malignant degeneration of colonic polyp.