婴儿纤维性错构瘤16例临床病理分析

发布时间：2018-02-13 07:37

本文关键词： 软组织肿瘤婴儿纤维性错构瘤免疫组织化学鉴别诊断　出处：《临床与实验病理学杂志》2017年07期 　论文类型：期刊论文

【摘要】：目的探讨婴儿纤维性错构瘤(fibrous hamartoma of infancy,FHI)的临床病理学特点、诊断及鉴别诊断。方法回顾性分析16例FHI的临床病理资料,并复习相关文献。结果 FHI男性10例,女性6例,年龄5天~3岁,平均1.11岁。肿瘤大小1 cm×0.5 cm×0.2 cm~11 cm×4 cm×4 cm不等,发生于上臂4例,背部3例,会阴2例,骶尾部、大腿、腋下、臀部、胸壁、小趾和足外侧各1例。肿瘤具有特征性的器官样结构,由富含胶原的纤维组织、原始间叶细胞岛及成熟的脂肪组织构成。免疫表型:纤维组织和原始间叶细胞vimentin均呈阳性,脂肪细胞S-100均呈阳性;10例纤维组织SMA呈阳性,8例间叶细胞CD34呈阳性,其中6例CD99呈阳性,Ki-67增殖指数均2%。结论 FHI是一种少见的发生于婴儿的皮下良性软组织肿瘤,具有特征性病理表现,结合发生部位、影像学资料、组织病理学特点及免疫表型可作出准确诊断。治疗以局部手术完整切除为主,对于肿物较大及多结节病例,建议扩大手术范围。
[Abstract]:Objective to investigate the clinicopathological features, diagnosis and differential diagnosis of fibrous hamartoma of infantile FHI.Methods the clinicopathological data of 16 cases of FHI were retrospectively analyzed and the relevant literatures were reviewed. Results there were 10 males and 6 females with FHI. The tumor size ranged from 1 cm 脳 0. 5 cm 脳 0. 2 cm~11 cm 脳 4 cm 脳 4 cm to 4 cm 脳 4 cm, including 4 cases of upper arm, 3 cases of back, 2 cases of perineum, sacrococcyx, thigh, armpit, buttocks, chest wall. The tumor was composed of collagen-rich fibrous tissue, primordial mesenchymal cell island and mature adipose tissue. Immunophenotype: vimentin was positive in fibrous tissue and primitive mesenchymal cell. S-100 of adipocytes were all positive in 10 cases of fibrous tissue SMA and 8 cases of mesenchymal cells were positive for CD34, among which 6 cases were positive for CD99 and Ki-67 proliferation index was 2%. Conclusion FHI is a rare subcutaneous benign soft tissue tumor occurring in infants. It can be accurately diagnosed by combining the location of occurrence, imaging data, histopathological features and immunophenotypes. It is suggested to expand the scope of the operation.
【作者单位】：福建医科大学省立临床医学院/福建省立医院病理科;福建医科大学省立临床医学院/福建省立医院药剂科;
【基金】：福建省自然科学基金(2015J01424)
【分类号】：R738.6

【相似文献】