尺骨和股骨原发软骨样副脊索瘤(附病例报告)

Primary chondroid parachordoma of ulna and femur:A report of two cases

目的探讨软骨样副脊索瘤的临床病理学特征及其与原发或转移性肿瘤的鉴别诊断。方法对2例原发于尺骨和股骨的软骨样副脊索瘤标本,通过光镜、免疫组化病理组织学观察和会诊及病理读片讨论会等方法进行研究。结果组织学特征为:肿瘤大部分由脊索瘤细胞和小部分软骨肉瘤细胞两种成分构成,呈大小不等的小叶状排列;小叶边缘为软骨样小梁或纤维性间隔;脊索瘤小叶内瘤细胞呈巢片状、小梁状或岛状排列,细胞大小不一,核较小而深染,核异型性不明显,胞质丰富呈大空泡状,间质为淡蓝色或粉染的粘液样物质;软骨样肉瘤小叶内可见具有软骨陷窝的双核和单核细胞,间质为透明的软骨基质和/或粘液样基质,构成了典型的软骨肉瘤图像。免疫组化(ABC法):EMA、CK、S-100蛋白、波形蛋白、NSE染色均呈阳性,Desmin、CD34均为阴性。会诊和读片讨论会均确诊为软骨样副脊索瘤。结论原发于尺、股骨的软骨样副脊索瘤非常罕见,常规HE染色很难确诊,免疫组化有助于明确诊断。

Objective To study the clinical pathological characteristic s and differentiation diagnosis of the chondroid parachordoma from primary tumors or metastatic to ulna and femur.Methods Two cases of primary chondroid parachordoma of the ulna and femur were studied by histopathological observation and immunohistochemical staining and by pathological consultation.Results The histological features of tumor were composed of two the chordoma cells subtotal and the chondrosarcoma cells of small-portio.The tumor was arranged vary in sizes of lobular and brink of lobular were seplum small girder of chondrioid,or the tissue fider.The tumor cells were distributed sheet,trabecula with or island-like in the intra-lobular,and the tumor cells were vary in size and the karyon were smaller with deep staining and the karyon heterotypic were no patent and the cytoplasm of rich shows large vacuole.The chondroidosaromas intra-lubular were viewed cells both-nucleus and uninuclear of chondrolacunae and the stroma were hyaline cartilage with or blennoid in the mesenchyma,and that picture of typical chondrosarcoma.EMA,CK,Vimtntin,S-100protein,NSE with immunohistochemical method(ABC)staining were positive and desmin,CD34were negative in the all of tumor cells.The chondroid para-chordoma was dignosed in the pathological read -section -conference and consultation.Conclusion Primary ulna and femur chondroid parachodoma is a rare in rate of neoplasm and is diagnosed difficultly.Immunohistochemical method would be helpful to diagnosis.