胸壁Askin瘤2例临床病理及免疫组化分析

目的探讨胸壁Askin瘤的临床病理和免疫组化特征。方法复习2例胸壁Askin瘤的临床病理资料,进行病理形态和免疫组化观察。结果男女患者各1例,年龄分别为22岁和35岁。临床主要表现为进行性增大的胸壁肿块。影像学特征为胸壁软组织致密影。镜检见肿瘤细胞为形态单一的小圆细胞,弥漫或巢状分布,1例可见典型的Homer_Wright菊形团结构,另1例局部区域可见明显的神经细胞分化。免疫组化:2例患者Syn、NSE和CD99均( );其中1例同时伴NF、vimentin和CK( ),EMA局灶性( )。结论Askin瘤是发生在胸肺区的一种少见的、高度恶性的、原始小圆细胞肉瘤,免疫组化Syn、NSE和CD99的阳性表达对Askin瘤的诊断和鉴别诊断具有重要参考价值。

Askin tumour of the chest wall: a clinicopathological and immunohistochemical analysis of 2 cases

Objective To explore the clinicopathological and immunohistochemical features of Askin tumor. Methods Two cases of Askin tumor on the chest wall were studied by histopathological observation and immunohistochemical staining. Results One of two cases was male, and another female. The age was 22 and 35 years respectively. The major symptoms of the patient were the chest wall mass enlarging progressively. The radiographic finding was a chest wall mass of soft_tissue density. Light microscopy showed an undifferentiated, small round_cell sarcoma. Homer_Wright rossetes were seen in one case, neurocyte differentiation were seen at some areas in another. Immunohistochemically, the tumor cells were positive for Syn,NSE and CD99, and positive for NF,vimentin,CK and EMA simultaneously in one case. Two cases died of the tumor metastasis for 12?m and 13?m respectively after operation. Conclusion Askin tumor is a rare, highly malignant, primitive small round_cell sarcoma arising from the thoracopulmonary region. Immunohistochemically, the expression of Syn,NSE and CD99 plays an important role in diagnosis and differential diagnosis of the Askin tumor.