骨外黏液样软骨肉瘤3例临床病理观察

Extraosseous myxoid chondrosarcoma:a clinicopathologic study of three cases

目的探讨骨外黏液样软骨肉瘤的临床病理特征、免疫表型、影像学特征及鉴别诊断。方法回顾性分析中国人民解放军总医院第四医学中心3例骨外黏液样软骨肉瘤的特征并进行文献复习。结果3例患者均为男性,年龄35~50岁,中位年龄44岁,肿瘤分别位于右大腿上端会阴部、右肩部、胸椎椎体及附件。镜检:肿瘤组织呈多结节状,瘤细胞大小及形态一致,呈圆形或稍长形,胞质嗜酸性,间质黏液样变性,部分细胞伴有上皮样及浆样分化。结论骨外黏液样软骨肉瘤是一种较为罕见的恶性软组织肿瘤,临床及影像学无明显特异性,确诊主要依靠病理组织学及分子遗传学等方法。

Objective To investigate the clinicopathological features,immunophenotype,imaging features and differential diagnosis of extraosseous myxoid chondrosarcoma.Methods The characteristics of 3 cases of extraskeletal myxoid chondrosarcoma in the Fourth Medical Center of the PLA General Hospital were analyzed retrospectively with review of the related literatures.Results The three patients were male,aged 35-50 years,with a median age of 44 years.The tumors were located in upper right thigh perineum,right shoulder,thoracic vertebrae and accessories thoracic.Microscopically,the tumor tissue was nodular,the tumor cells were consistent in size and shape,round or slightly long,the cytoplasm was eosinophilic,interstitial myxoid degeneration,and some cells were epithelioid and plasmacytoid differentiation.Conclusion Extraskeletal myxoid chondrosarcoma is a rare malignant soft tissue tumor.There is no obvious specificity in clinical and imaging.The diagnosis mainly depends on histopathology and molecular genetics.