婴儿胸壁错构瘤5例临床病理学分析

Mesenchymal hamartoma of the chest wall in infants:a clinicopathologic study of five patients

目的分析婴儿胸壁错构瘤的临床病理学特征、影像学特征、以及诊断与鉴别诊断,旨在提高临床医生对该罕见疾病的认识。方法对5例发生于婴儿的胸壁错构瘤的临床特征、病理组织学特点及免疫表型进行分析,并复习相关文献。结果5例患者均表现为逐渐增大的胸壁肿块,常伴呼吸道症状,男女比例为3∶2,左侧与右侧比例为3∶2,3例患儿累及一根肋骨,2例患儿病灶累及多根肋骨,中位年龄7个月。CT表现为来源于肋骨的肿块呈膨胀、筛网状结构,肿块内密度不均,呈不规则钙化密度影及软组织团块影。病理学大体上表现为囊性与实性相间的肿块,囊性区可有黏液、出血。镜下瘤组织实性区由透明软骨组织、胶原纤维组织增生为主,可见软骨内成骨;出血性囊性区见动脉瘤样骨囊肿样结构。所有标本中以上成份均以不同比例呈现,免疫组化可提示病变各组织成分来源。5例患者均行手术完整切除治疗。结论婴儿胸壁错构瘤是一种临床非常罕见的良性疾病,术前常被误诊为恶性肿瘤,具有特殊的临床病理学特征,治疗以手术完整切除为主。

Objective To explore the clinicopathologic and radiological characteristics,diagnosis and differential diagnosis of chest wall hamartoma in infants.Methods The clinical characteristics,histopathologic features and immunophenotype of five chest wall hamartomas were observed and relevant literature were reviewed.Results Five patients were gradually enlarged as chest wall masses,often accompanied by respiratory symptoms.The male/female ratio was 3∶2,the ratio of left to right was 3∶2.Multiple ribs were involved in 2 cases,One rib was involved in 3 cases,and the median age was 7 months.CT imaging showed that the mass from the rib was expanded and cribriform,with uneven density,irregular calcification density shadow and soft tissue mass shadow.Microscopically,the solid part of the tumor tissue was mainly composed of hyaline cartilage and collagen hyperplasia,and osteogenesis was seen in the cartilage;the aneurysmal bone cyst-like structure was noted in the hemorrhagic fluid-filled area.Immunohistochemistry indicated the origin of the components of the lesion.All the 5 patients were treated with complete resection.Conclusion Infant chest wall hamartoma is a very rare clinical benign disease.Preoperative diagnosis is often mistaken as malignant tumor.It has characteristic clinicopathologic features.The main treatment is complete surgical excision.