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目的探讨异位错构瘤性胸腺瘤的临床病理学特征、免疫学表型和组织发生。方法对2例异位错构瘤性胸腺瘤进行临床病理学分析,采用免疫组织化学LSAB法行AE1/AE3、细胞角蛋白(CK)5、CK7、CK8、CK20、上皮膜抗原(EMA)、波形蛋白、CD5、CD10、α-平滑肌肌动蛋白(SMA)、calponin、结蛋白、CD34、S-100蛋白、CD57、胶质纤维酸性蛋白(GFAP)、甲状腺转录因子(TTF)-1和CD99标记。结果两例均发生于男性,年龄分别为20岁和40岁,因发现胸骨柄上缘肿块6个月和左锁骨上肿块2个多月就诊。肿块呈球形和卵圆形,边界清楚,最大直径分别为5cm和3cm,切面呈灰白色,质地柔软。组织学上,肿瘤由梭形细胞、上皮细胞和成熟的脂肪细胞3种成分混合组成。梭形细胞成分在2例中分别占到85%和70%,形态上类似纤维母细胞,多呈束状、编织状或席纹状排列。上皮细胞成分在两例中均占10%左右,形态上以非角化性鳞状上皮为主,可呈实性的小岛屿状、类似造釉细胞瘤的条索状和扩张的囊肿样排列,部分区域显示腺样分化,可见腺管形成。上皮细胞和梭形细胞在形态上有移行。成熟的脂肪组织呈不规则性分布,在两例肿瘤中所占的比例分别为<5%和20%。免疫组织化学标记显示,上皮细胞表达AE1/AE3、CK5、CK7、CK8和EMA,梭形细胞除CK外还表达波形蛋白、CD10和CD34,并部分表达α-SMA和calponin。两种细胞成分均不表达CK20、CD5、TTF-1、结蛋白、S-100蛋白、CD57、GFAP和CD99。结论异位错构瘤性胸腺瘤是一种好发于中青年男性锁骨上和胸锁关节附近软组织内的良性肿瘤,是一种由上皮和肌上皮组成的混合性肿瘤,可能起自于His颈窦,采用鳃原基混合瘤来命名或许更为合适。
Objective To investigate the clinicopathological features, immunological phenotypes and histopathology of ectopic hamartoma. Methods Two cases of ectopic hamartomatous thymoma were analyzed by immunohistochemical method. The expressions of AE1 / AE3, cytokeratin 5, CK7, CK8, CK20, epithelial membrane antigen (EMA) Vimentin, CD5, CD10, SMA, calponin, desmin, CD34, S-100 protein, CD57, glial fibrillary acidic protein (GFAP), thyroid transcription factor (TTF) mark. Results Both cases occurred in males, aged 20 and 40 years old, respectively. The upper suture of the sternum was found for 6 months and the left supraclavicular mass for more than 2 months. Mass was spherical and oval, clear boundary, the maximum diameter of 5cm and 3cm, the section was gray, soft texture. Histologically, the tumor consists of a mixture of spindle cells, epithelial cells and mature adipocytes. Spindle cells accounted for 85% and 70% of the 2 cases, respectively. Morphology was similar to that of fibroblasts, mostly in the form of bundles, weaves or stripes. Epithelial cell composition in both cases accounted for about 10% morphologically non-keratinized squamous epithelium may be solid small island-like, like ameloblastoma cord-like and expansion of the cyst-like arrangement , Some areas showed adenoid differentiation, visible duct formation. Epithelial cells and spindle cells in the morphology of migration. Mature adipose tissue was irregularly distributed, accounting for <5% and 20% of the two tumors, respectively. Immunohistochemical staining showed that epithelial cells expressed AE1 / AE3, CK5, CK7, CK8 and EMA. In addition to CK, spindle cells expressed vimentin, CD10 and CD34, and partially expressed α-SMA and calponin. Neither cell component expressed CK20, CD5, TTF-1, desmin, S-100 protein, CD57, GFAP, and CD99. Conclusions Ectopic hamartoma is a benign tumor that occurs in the soft tissues of the supraclavicular and sternoclavicular joints in young and middle-aged men. It is a mixed tumor consisting of epithelial and myoepithelial epithelium that may start from His Cervical sinus, gill primordial mixed tumor to name may be more appropriate.