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目的探讨隆凸性皮肤纤维肉瘤(DFSP)的诊断和治疗方法。方法分析第二军医大学长海医院收治的DFSP病人11例,均由手术后病理确诊。其中DFSP纤维肉瘤型(DFSP-FS)4例。肿瘤位于躯干部9例,位于四肢2例。2例行局部常规切除,其余9例均行扩大切除术。3例在术后行放射治疗,平均剂量55Gy(50~65Gy)。结果切除11例标本,CD34阳性10例,阴性1例。术后随访1~10年(平均4.3年),失访1例。复发6例,平均复发时间2.6年(10个月至5年)。11例均未见有远处转移,无死亡。结论DFSP在临床上易与其他软组织肿瘤相混淆。诊断主要依据病理。治疗DFSP最主要的方法是扩大的手术切除,切缘阳性或肿块太靠近切缘的辅以放疗。单独使用放疗可以作为偶见的无法切除肿块的治疗方式。
Objective To investigate the diagnosis and treatment of Congenital Dermatofibrosarcoma (DFSP). Methods 11 cases of DFSP patients admitted to Changhai Hospital of Second Military Medical University were analyzed by pathology. Including DFSP fibrosarcoma type (DFSP-FS) in 4 cases. Tumors located in the trunk of 9 cases, located in 2 limbs. 2 routine routine local excision, the remaining 9 cases underwent expansive resection. Three patients underwent radiotherapy after the operation, with an average dose of 55 Gy (50-65 Gy). Results 11 cases were resected, 10 cases were CD34 positive and 1 case negative. The patients were followed up for 1 to 10 years (average 4.3 years) and lost to one case. 6 cases relapsed, the average recurrence time of 2.6 years (10 months to 5 years). None of the 11 patients had distant metastasis and no death occurred. Conclusion DFSP is easily confused with other soft tissue tumors in clinic. The diagnosis is mainly based on pathology. The main method of treatment of DFSP is to expand the surgical resection, the positive margin or the mass is too close to the margins supplemented with radiotherapy. Radiation alone can be used as an occasional method of treatment of unresectable lumps.