13例Askin瘤诊断与治疗

Diagnosis and Treatment of 13 Askin's Tumor Cases

目的:分析Askin瘤的临床病理特征,探讨该肿瘤的诊断及治疗。方法:回顾性分析天津医科大学附属肿瘤医院1997年1月～2008年1月13例Askin瘤患者的临床、病理及随访资料。13例患者均见胸壁肿物,其中6例发生肺部浸润。13例患者均接受了化疗及病灶局部的放疗,6例行姑息性肿瘤切除术,3例接受了肿瘤根治术。结果:13例患者的中位总生存期(OS)为16个月,临床表现主要为胸闷、咳嗽、憋气及胸壁进行性生长的疼痛性肿块;组织学上以稀少的纤维血管间质将小圆、卵圆瘤细胞围绕成巢状或小叶状结构,网染细胞间网状纤维少或无,PAS染色部分病例(+),CD99、VTM部分病例(+),此外,还可表达NSE、S-100。结论:Askin瘤预后差,手术切除是治疗的主要方法,主张以手术为主的综合治疗。尽可能的切除肿瘤,并进行规范的放化疗,能够延长患者的生存期。

Objective： To analyze the clinical and pathological characteristics and to discuss the diagnosis and treatment of Askin＇s tumor. Methods： Thirteen cases of Askin＇s tumor were collected between January 1997 and January 2008 in Tianjin Medical University Cancer Institute and Hospital and analyzed retrospectively. Chest wall masses were demonstrated in all 13 cases, and 6 pa- tients had lung involvement. All the cases received chemotherapy and local radiation, six received palliative tumorectomy, and three re- ceived radical surgery. Results： The median overall survival of the 13 patients was 16 months. The clinical characteristics were chest distress, cough, breath holding, and growing tumors in the chest wall. Small round cells were observed in the tissue samples under mi- croscopy. The tumor ceils were classified into different shape based on the interstitial tissue. Little reticular fibers were observed among the tumor cells. PSA, CD99, and VIM were positive in some cases. In addition, they also expressed NSE and S-100. Patients who under- went surgery with chemotherapy and radiation had longer survival than those treated with chemotherapy and radiation. Conclusion： Askin＇s tumors have poor prognosis. Surgery with chemotherapy and local radiation are superior to other regimens.