腹壁韧带样纤维瘤的诊断与临床病理分析

The diagnosis and treatment of desmoid tumors of abdominal wall

目的总结腹壁韧带样纤维瘤的诊断及治疗特点。方法收集经手术病理证实为腹壁韧带样纤维瘤的21例患者的临床资料,包括临床症状、包块特征、影像学资料、病理(常规及免疫组化)、手术及术后辅助治疗等,并进行回顾性分析。结果所有病例均有明确的手术、外伤史或妊娠史,而无明显症状,包块平均直径4.5cm,女性18例。MRI提示边界不清,T1WI常为等信号或稍低信号,T2WI提示稍高信号,增强后明显强化。肿瘤位于肌肉内或与筋膜相连处,质地坚韧,由梭形细胞和胶原纤维束组成,胶原纤维成分特别丰富,无明显包膜,边缘不清,向周围组织浸润,常侵及横纹肌,无病理性核分裂象。免疫组化检查梭形细胞波形蛋白(Vimentin)5例均阳性反应;平滑肌抗体SMA灶性阳性5例;ki-67指数均阴性或不高于5%。术中需切除周围2～3cm组织,手术后局部复发2例。结论腹壁韧带样纤维瘤是一种交界性肿瘤,临床及病理有一定的特征,为其诊断及治疗提供依据。

Objective To summarize the diagnosis and treatment of desmoid tumor of abdominal wall. Methods Retrospectively analysis 21cases of desmoid tumor of abdominal wall about clinical and histopathologic findings, and the treatment results. Results There had no obvious symptoms, and had history of surgery, trauma or pregnancy in all cases. The mean diameter of tumor was 4.5era. There were 18 female cases. There had obscure boundary on MRI. Compare to the signal intensity of muscle,all cases were isointense or slightly hypointese on TlWI, and slightly hyperintense on T2WI. Tumors located in muscles or around fascia,and its composition were spindle cells and collagen fibers. There had no obviously envelope and boundary, and had tissue infiltration, no pathological mitotic figure. In immunohistochem- isty, 5 cases had positive reaction in vimentin, five positive in SMA, and the figure of ki-67 were negative or on more than 5%. Need to excise the tumor and the tissues surrounding in 2-3cm. There had local recurrence in two cases. Conclusion There arc certain clinical and histopathologie regularities of desmoid tumor of abdominal wall, and these are helpful in diagnosis and treatment of desmoid tumor.