Zhang N, Gao Y, Li X H, Zhou J, He L J
Department of Pathology, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
Department of Pathology, Kunming Children's Hospital, Kunming 650228, China.
Zhonghua Bing Li Xue Za Zhi. 2018 Sep 8;47(9):706-709. doi: 10.3760/cma.j.issn.0529-5807.2018.09.011.
To study the clinicopathologic features, diagnosis and differential diagnosis of chest wall hamartoma of infancy. The clinical data, histopathologic features and immunophenotype of five chest wall hamartomas were analyzed, and the relevant literature was reviewed. 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. Chest wall hamartoma is a rare but benign lesion of infancy, showing characteristic clinicopathologic features. The prognosis are excellent after complete surgical excision.
研究婴儿胸壁错构瘤的临床病理特征、诊断及鉴别诊断。分析5例胸壁错构瘤的临床资料、组织病理学特征及免疫表型，并复习相关文献。切除时患者年龄为7个月零8天至20个月，尽管病变在新生儿期即被发现。男女比例为4∶1。3例为单侧，2例有多发病变；4例位于右侧。胸部CT扫描显示膨胀性软组织肿块，密度不均匀，含钙化或骨化，累及一根或多根肋骨，显示部分骨质破坏。所有患者均行完整切除。大体上，病变由实性和囊性区域组成，后者可见出血。显微镜检查显示实性区域由多叶状透明软骨岛组成，散布于梭形或椭圆形间充质细胞、波浪状骨和胶原纤维之间。在软骨样组织周围，有局灶性软骨内成骨和黏液样背景。此外，有由出血性扩张的囊性间隙形成的动脉瘤样骨囊肿样改变区域。免疫组化一般对诊断无必要。胸壁错构瘤是婴儿期一种罕见的良性病变，具有特征性的临床病理特征。完整手术切除后预后良好。
