婴儿胸壁错构瘤临床病理学观察

Chest wall hamartoma of infancy： a clinicopathologic observation of five cases

目的探讨婴儿胸壁错构瘤的临床病理特征、诊断及鉴别诊断，以提高对该少见病变的认识。方法收集北京儿童医院2005年1月至2017年11月间5例婴儿胸壁错构瘤的临床资料、病理组织学特征和免疫表型进行分析，并复习相关文献。结果5例患者手术时年龄7个月8天至1岁8个月，但患儿均为生后即发现胸壁包块，男女比为4：1，右侧与左侧比为4：1，其中3例为单侧单发病变，2例为单侧多发病变。CT显示肋骨膨胀性及筛网样改变，其内见钙化及骨化。5例患儿均采取手术完整切除的治疗方案。送检病理标本最大径3-11cm，囊实相间，囊腔内见凝血或血液。镜下实性区为软骨、编织骨、梭形纤维母细胞及胶原纤维各种成分不同比例的混合，可见软骨内成骨，囊性区为继发性动脉瘤样骨囊肿成分，部分病例局灶见蓝染黏液。免疫组织化学染色提示病变内各成分来源，但对诊断意义较小。结论婴儿胸壁错构瘤是一种罕见的良性病变，具有独特的临床病理学特点．预后良好。

Objective To study the clinicopathologic features, diagnosis and differential diagnosis of chest wall hamartoma of infancy. Methods The clinical data, histopathologic features and immunophenotype of five chest wall hamartomas were analyzed, and the relevant literature was reviewed. Results The patients＇ age ranged from seven months and eight days to 20 months at time of resection, although the lesions were detected in the neonatal period. The male-to-female ratio was 4： 1. Three cases were unilateral, two presented with multiple lesions ; and four were located on the right side. CT-scan of the chest showed expansile soft tissue masses with heterogeneous density containing calcifications or ossifications in one or more ribs showing partial bone destruction. Patients were all treated by complete resection. Grossly, the lesions comprised solid and cystic areas, the latter showing hemorrhage. Microscopic examination revealed the solid areas were composed of islands of multilobulated hyaline cartilage interspersed within spindle or oval mesenchymal cells, waved bone and collagen fibers. Around the chondroid tissue, there were focal endochondral ossification and mucus-like background. In addition, there were areas of aneurysmal bone cyst-like changes formed by hemorrhagic dilated cystic spaces. Immunohistochemistry was generally not necessary to establish the diagnosis. Conclusions Chest wall hamartoma is a rare but benign lesion of infancy, showing characteristic clinieopathologic features. The prognosis are excellent after complete surgical excision.