磷酸盐尿性间叶性肿瘤的临床病理观察

Phosphaturic mesenchymal tumor: a clinicopathologic analysis

目的探讨磷酸盐尿性间叶性肿瘤(PMT)的临床病理学特征。方法对1例发生于肋骨的磷酸盐尿性间叶性肿瘤进行临床资料分析、组织病理学观察和免疫组化标记。结果肿瘤细胞梭形,呈束状或编织状排列,细胞核圆形或卵圆形,无明显异型性及核分裂。间质内可见黏液样、软骨、骨样基质及成熟脂肪组织,血管丰富,且伴有不规则钙盐沉积。免疫组化:瘤细胞vimentin、CD56、NSE和CD99(+),CD68散在(+);AE1/AE3、desmin和S-100均(-)。结论磷酸盐尿性间叶性肿瘤是一种罕见的表现良善的间叶性肿瘤,诊断依赖于组织病理学、免疫组化标记并结合临床资料才能正确诊断。治疗应予以完整切除。

Purpose To study the clinicopathological features of the phosphaturic mesenchymal tumors. Methods The clinicopathological data,histology and immunohistochemical findings of 1 case of phosphaturic mesenchymal tumor were analyzed with review of the relevant literature. Results The tumor was primarily composed of spindle cells,and accompanied with osteoclast-like giant cells. Their background were rich in blood vessels. On immunohistochemical study,the tumor cells were positive for vimentin,CD56,NSE and CD99. Conclusioin The phosphaturic esenchymal tumors are either benign or low-grade malignant. Their histomorphology are variable and short of specificity. It is important to make the correct diagnosis in combination with their common features and clinical data.