纤维肉瘤型隆突性皮肤纤维肉瘤8例临床病理学观察

发布时间：2018-07-24 12:27

【摘要】：目的探讨纤维肉瘤型隆突性皮肤纤维肉瘤(fibrosarcomtous dematofibrosarcom protuberans,FS-DFSP)的临床病理学特征及鉴别诊断。方法回顾性分析8例FS-DFSP的临床资料、组织学形态和免疫表型。结果 8例中男性6例,女性2例,平均年龄42岁,中位年龄44岁,年龄27~55岁。肿瘤部位:2例位于腰背部,2例位于肩部,2例位于大腿,前额部、枕部各1例。临床表现与经典型DFSP相似,表现为皮下缓慢性生长的无痛性肿块,其中4例短期内生长迅速。8例中7例为原发性,1例为复发性。肿瘤直径2.0~7.5cm,平均4.3 cm。镜检:肿瘤境界不清,呈浸润性生长,肿瘤组织除查见经典型DFSP区域外,还可见FS区域。免疫表型:所有病例经典型DFSP区域瘤细胞均表达CD34,FS区域瘤细胞表达CD34明显减弱。随访结果:8例中5例局部复发。结论 FS-DFSP是DFSP常见亚型之一,FS-DFSP的临床、病理和生物学行为均与经典型DFSP明显不同,具有较高的复发率和转移率。
[Abstract]:Objective to investigate the clinicopathological features and differential diagnosis of fibrosarcoma Carina dermatofibrosarcoma (fibrosarcomtous dematofibrosarcom protuberansa FS-DFSP). Methods the clinical data, histological morphology and immunophenotype of 8 cases of FS-DFSP were retrospectively analyzed. Results there were 6 males and 2 females with an average age of 42 years, with a median age of 44 years and an age of 2755 years. Tumor site: 2 cases were located in the back of the waist, 2 cases were located in the shoulder, 2 cases were located in the thigh, 1 case in the forehead and 1 case in the occipital part. The clinical manifestations were similar to those of classical DFSP and showed subcutaneous slow growth painless masses, of which 7 cases were recurrent in 7 out of 4 cases with rapid growth in a short period of time. The diameter of the tumor was 2.0 ~ 7.5 cm, with an average of 4.3 cm. Microscopic examination: tumor boundary is not clear, showing infiltrative growth, tumor tissue can be found in addition to classical DFSP area, also can be seen in FS region. Immunophenotype: the expression of CD34 in CD34 / FS region was significantly decreased in all cases of classic DFSP region tumor cells. Follow-up results showed that 5 of 8 cases had local recurrence. Conclusion FS-DFSP is one of the common subtypes of DFSP. The pathological and biological behaviors of FS-DFSP are obviously different from those of classical DFSP, and the recurrence rate and metastasis rate are higher.
【作者单位】：广西医科大学附属南宁市第一人民医院病理科;
【分类号】：R739.5

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