探讨磷酸盐尿性间叶瘤的临床病理学特点。

回顾分析10例患者的临床资料，其中男性患者6例，女性患者4例；年龄范围23～55岁；患者均有1～13年骨痛、关节痛和活动困难的病史。观察10例磷酸盐尿性间叶瘤的形态和免疫表型。

检查发现患者血磷低、尿磷高；肿瘤最大径0.80 cm～5.00 cm，瘤组织为间叶组织来源，可见多少不等的梭形纤维母细胞样细胞、脂肪细胞、软骨样细胞、黏液样细胞等，瘤组织富于血管。4例病变中有少见的絮状或不规则砂砾样钙盐沉积；3例发生于软组织的肿瘤周边见骨壳形成；8例细胞分裂象少见；2例核分裂象多见并且异型性明显；10例瘤细胞波纹蛋白(VIM)及CD56均阳性，7例神经元特异性烯醇化酶(NSE)阳性，3例B细胞淋巴瘤/白血病-2(Bcl-2)瘤细胞阳性，结蛋白、S-100、角蛋白单克隆抗体(AE1/AE3)均阴性，8例Ki-67指数为1%～5%，仅2例为20%～30%。

磷酸盐尿性间叶瘤多为良性或低度恶性的间叶组织肿瘤，因组织学多种多样而易被误诊，掌握其共同的特征并结合临床资料方能正确诊断。

To study the clinicopathologic features of phosphaturic mesenchymal tumor.

The clinical and pathologic data of ten cases with phosphaturic mesenchymal tumor were evaluated, in the ten patients, six were males and four were females.Their age at the time of operation ranged from 23 to 55 years old.All cases had a history of chronic bone pain, arthralgia, limitation in movement for 1 to 13 years.Morphology and immune phenotype of phosphaturic mesenchymal tumor were observed.

Hypophosphatemia and byperphosphaturia were found in all patients.The diameters of tumor ranged from 0.8 cm to 5.0 cm.Microscopically, the tumors were composed of various mesenchymal cells, including spindled fibroblast-like cells, adipocytes, chondroid cells and mucinous cells, the tumor cells were rich in blood vessels.Four cases were also dystrophic calcification with an unusual flocculent or "grungy" pattern.Bone shell was formed surrounding the tumors which occured in soft tissue in three cases.Mitotic figures were rare in eight cases.In two cases however, mitotic figures and bizarre cells were commonly encountered.On immunohistochemical study, the tumor cells were all positive to vimentin and CD56.NSE was positive in seven and Bcl-2 was positive in three cases.The staining for desmin, S-100 and AE1/AE3 was negative.Ki67 proliferation index was 1%~5% in eight cases and 20%~30% in two case.

Most of the phosphaturic mesenchymal tumors are either benign or low grade malignant mesenchymal tumors.They can be mistaken as other neoplasms due to the morphologic heterogeneity.Clinical features and laboratory investigation results are helpful correct diagnosis phosphaturic mesenchymal tumor.