Abstract:ObjectiveTo study the clinicopathologic features of phosphaturic mesenchymal tumor.MethodsThe 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.ResultsHypophosphatemia and byperphosphaturia were found in all patients.The diameters of tumor ranged from 0.8cm to 5.0cm.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.ConclusionsMost 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.
[1]Folpe AL,FanburgSmith JC,Billings SD,et al.Most osteomalaciaassociated mesenchymal tumors are a single histopathologic entity:an analysis of 32 cases and a comprehensive review of the literature[J].Am J Surg Pathol,2004,28(1):1-30.
[2]钟定荣,刘彤华,杨堤,等.骨软化或佝偻病相关的间叶组织肿瘤临床病理分析[J].中华病理学杂志,2005,34(1):724-728.
[3]聂秀,邓仲端,杨秀萍,等.软组织磷酸盐尿性间叶肿瘤的临床病理分析[J].临床与实验病理学杂志,2007,23(5):557-561.
[4]De Beur SM,Finnegan RB,Vassiliadis J,et al.Tumors associated with oncogenic osteomalacia express genes important in bone and mineral metabolism[J].J Bone Miner Res,2002,17(6):1102-1110.
[5]Bowe AE,Finnegan R,Jan de Beur SM,et al.FGF-23 inhibits renal tubular phosphate transport and is a PHEX substrate[J].Biochem Biophys Res Commun,2001,284(4):977-981.
[6]Weidner N,Santa Cruz D.Phosphaturic mesenchymal tumors:a polymorphous group causing osteomalacia or rickets[J].Cancer,1987,59(8):1442-1454.
[7]Ogose A,Hotta T,Emura I,et al.Recurrent malignant variant of phosphaturic mesenchymal tumor with oncogenic osteomalacia[J].Skeletal Radio,2001,30(2):99-103.
[8]杨志寅.现代医学科学发展中的缺憾与思考[J/CD].中华诊断学电子杂志,2013,1(1):1-7.