磷酸盐尿性间叶瘤临床病理观察  

Phosphaturic mesenchymal tumor: a clinicopathological analysis and review of literature

作　　者：赵敏[1] 陆江阳[1] 王宏伟[1] 金贻铎[1] 康佳蕊[1] 

机构地区：[1]解放军总医院第一附属医院(304医院)病理科,北京100048

出　　处：《诊断病理学杂志》2016年第3期202-205,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的通过对磷酸盐尿性间叶瘤(PMT)的临床与病理观察及复习文献,探讨其病变机制与病理特点。方法对1例骨病患者的右大腿肿块切除标本进行光镜、电镜观察和免疫组化检测,结合临床资料,并复习相关文献。结果患者表现为持续性全身多处骨痛、低磷酸血症、高磷酸盐尿和高血清碱性磷酸酶,血钙正常。临床行右大腿肿块切除,术后5天,血磷恢复正常。大体见灰黄、灰红色肿物,大小3 cm×2.5 cm×2 cm,质中,与周围界限不清。瘤组织主要由片状分布的圆形细胞和短梭形细胞构成,胞质丰富红染,境界不清,局部可见黏液变。间质见血管外皮瘤样细小血管、厚壁静脉血管,散在脂肪岛,局部灶性出血及多核巨细胞浸润,肿瘤边缘近乎完整的薄层纤维包膜。免疫组化显示肿瘤细胞vimentin、CD68、HMB-45、CD63和FGF23强(+)。电镜见瘤细胞胞质内含数量不等的高电子密度的致密物质,部分颗粒与初级溶酶体关系密切。结论 PMT有特征性的临床表现,但缺乏特异的形态学改变,一般应结合临床检查作出最后诊断。本例形态学主要呈血管平滑肌脂肪瘤样病理改变,HMB-45(+),是该瘤一个新的病理学特点,结合瘤细胞对FGF23高表达及电镜见斑片状致密物,对诊断有重要意义。Purpose To study the clinicopathological characteristics of the phosphaturic mesenchymal tumor,mixed connective tissue variant（ PMT / MCT）. Methods The tumor resected from the right thigh of a patient with pantalgia was studied by light and electron microscopy,real-time PCR and immunohistochemistry,and the related literature was reviewed. Results The typical clinical symptom was intractable pantalgia characterized by hypophosphatemia,phosphaturia,elevated serum alkaline phosphatase,and normal level of serum calcium. Five days after removal of the tumor,the serum phosphate level returned to normal. Microscopically,the tumor was primarily composed of numerous oval and plump spindle cells with red and broad cytoplasm,and it was rich in vessels,including hemangiopericytoma like vessels and thick-walled blood vessels. In addition,there were scattered islands of mature adipose tissue,hemorrhage and obvious mucinous degeneration were observed as well. The tumor had no clear border in gross appearance,but an incomplete rim of fibrous membrane could be seen through microscope. Under electronmicroscope,microgranular and lamellar electron dense matter was seen in cytoplasm,some of which were combined with lysosome. The tumor cells expressed Vim,CD68,HMB-45 and FGF23,whereas they were negative for all other markers. Conclusions Phosphaturic mesenchymal tumors have distinguished clinical characteristics,but lack of specific morphological changes. In general,we should make the final diagnosis according to the clinical examination and laboratory data. The tumor may be confused with angiomyolipoma because of the angiomyolipoma-like pleomorphic appearances. The tumor cells are positive for HMB-45,in addition high expression of FGF23,and lamellar electron dense matter in cytoplasm under electron microscope. We conclude that these are new pathological features of these tumors. Our results show certaim significance for the diagnosis of phosphaturic mesenchymal tumors.

关 键 词：磷酸盐尿性间叶瘤 骨软化症 FGF23 电子显微镜 

分 类 号：R738.6[医药卫生—肿瘤]