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目的探讨成人胸壁软骨间叶性错构瘤的临床病理和影像学特征、诊断与鉴别诊断及治疗。方法观察2例发生于成人的胸壁软骨间叶性错构瘤的临床及影像学表现、组织学形态及免疫组化特征,并复习相关文献。结果2例患者男女各1例,年龄均为18岁。肿瘤分别位于胸壁右侧第10肋和右侧第4肋骨,均因体检发现肋骨膨胀伴有钙化的肿块而就诊。镜下见实性区主要为分化成熟的透明软骨,部分区域见不成熟软骨母细胞样细胞和破骨细胞样多核巨细胞;梭形细胞为增生的纤维母细胞以及胶原纤维。囊性区域呈典型动脉瘤性骨囊肿特征,囊腔内充满血液,囊壁和囊内分隔组织内含纤维母细胞、破骨细胞样多核巨细胞和反应性新生骨,无内皮细胞覆盖。免疫组化:vimentin(+),CD56、calponin、caldesmon、S-100、SMA、desmin、CD34和SOX-10(-)。患者均手术切除肿块及患侧肋骨。结论胸壁软骨间叶性错构瘤发生于成人非常罕见,需结合临床和影像学及病理组织学进行诊断与鉴别诊断。手术完整切除肿块可治愈。
Objective To investigate the clinicopathological and imaging features, diagnosis, differential diagnosis and treatment of adult chest wall mesenchymal hamartoma. Methods The clinical and imaging features, histological features and immunohistochemical features of 2 cases of chest wall cartilage myxoma occurred in adults were observed. The related literatures were reviewed. Results Two patients were male and one female, both of whom were 18 years old. Tumors were located on the right side of the thoracic rib 10 and the fourth rib on the right, both because of physical examination found rib expansion with calcified mass and treatment. Microscopically, the real area was mostly mature hyaline cartilage, immature chondroblastoid cells and osteoclast-like multinucleated giant cells in some areas, and fibroblasts and collagen fibers in spindle cells. The cystic area is characterized by typical aneurysmal bone cysts. The cyst is filled with blood. The cyst wall and cystic septum contain fibroblasts, osteoclast-like multinucleated giant cells and reactive new bone without endothelial cells. Immunohistochemistry: vimentin (+), CD56, calponin, caldesmon, S-100, SMA, desmin, CD34 and SOX-10 (-). Patients were surgically removed lumps and ipsilateral ribs. Conclusions The chest wall cartilage lobe hamartoma is very rare in adults and needs to be diagnosed and differentiated with clinical and imaging histopathology. Complete removal of the tumor can be cured.