肌纤维瘤30例临床病理分析
作者: |
1储婧,
1王一真,
1方园
1 安徽省儿童医院病理科,合肥 230069 |
通讯: |
储婧
Email: chujing198603@163.com |
DOI: | 10.3978/j.issn.2095-6959.2020.03.009 |
摘要
目的:探讨肌纤维瘤的临床病理特征、诊断及鉴别诊断。方法:回顾性分析2012至2019年就诊于安徽省儿童医院的30例经手术切除诊断为肌纤维瘤的患者的临床病理特征、治疗和预后。结果:30例患者;男16例,女14例,年龄42 h~10岁,诊断时的中位年龄为15.5个月。均为单发孤立性包块。出生时即发现包块的9例,发生于头颈部10例,四肢8例,躯干11例,1例发生于末端回肠肠壁内。18例肿物位于真皮及皮下浅层,8例位于深部肌肉内,3例侵犯额骨,1例位于肠壁内。镜检显示梭形肿瘤细胞呈现典型的带状,其特征是周围细长细胞排列成短束或漩涡状,中心圆形至多边形细胞围绕薄壁不规则分支血管形成血管外周细胞瘤样结构。细胞无明显异型性,核分裂象罕见。免疫表型:肿瘤细胞表达Vimentin和SMA,不表达S-100,Desmin,CD34,CK,CD99,CD117,β-catenin,ALK,STAT6和GRIA2,Ki-67分析显示低增值率(<5%)。所有患者经外科手术完整切除瘤体。术后随访1~798个月,除3例失访,其余患者均存活,且无复发。结论:肌纤维瘤是婴幼儿少见肿瘤,预后良好。综合临床、组织病理学和免疫组织化学标志对于正确诊断是必须的。
关键词:
肌纤维瘤;病理诊断;鉴别诊断
Clinical pathological analysis of 30 cases of myofibrils
CorrespondingAuthor: CHU Jing Email: chujing198603@163.com
DOI: 10.3978/j.issn.2095-6959.2020.03.009
Abstract
Objective: To explore the clinical pathological features, diagnosis and antidiastole of myofibrils. Methods: Regression analysis is engaged to explore the clinical pathological features, treatment and prognosis of 30 diagnosed myofibrils excised by operation in Anhui Provincial Children’s Hospital in 2012–2019. Results: The participants of these 30 cases consisted of 16 males and 14 females, whose ages ranged from 42 hours to 10 years old, and the median age is 9.5 months. 9 cases of mass found at birth. The number of head-and-neck cases, appendicular cases truncal ileum-end case was 10, 8, 11 and 1 respectively. Among them, 18 were located in dermis and superficial subcutaneous layer, 8 in deep muscle, 3 in frontal bone and 1 in intestinal wall. Spindle tumor cells that presented typical band was characterized by short and circinate threadlike cells surrounding, and that rounded and polygonal cells embracing thin-wall irregular branch vessel form peripheral blood vessel cell cancer structure in the center. There were no obvious atypia and rare nuclear fission in cells. Types of immunity: cancer cells expressed Vimentin and SMA, but non-expressed S-100, Desmin, CD34, CK, CD99, CD117, β-catenin, ALK, STAT6 and GRIA2. Analysis of Ki-67 showed low growth rate (which is less than 5%). The tumors of all the patients were cut completely by surgical operation. During the 1 to 798 months of follow-up, except 3 cases which were not follow-up, the other patients survived and didn’t relapse. Conclusion: Myofibrils are rarely seen in infants and its prognosis is favorable. Correct diagnosis is inseparable from clinical, histopathology and immunohistochemical markers.
Keywords:
myofibrils; pathological diagnosis; differential diagnosis