Bridge J A, DeBoer J, Travis J, Johansson S L, Elmberger G, Noel S M, Neff J R
Department of Pathology, University of Nebraska Medical Center, Omaha.
Am J Pathol. 1994 Feb;144(2):215-20.
Cytogenetic analysis of four specimens (biopsy, definitive surgical, and two separately occurring lung metastases) of a dedifferentiated chondrosarcoma with a rhabdomyosarcomatous component revealed clonal karyotypic abnormalities in each. Anomalies seen in all specimens included a structurally aberrant chromosome 17 and extra copies of chromosomes 5, 7, 12, and 20. The derivation of the chromosomally abnormal cells was determined by a combined immunocytochemical/cytogenetic approach that allowed simultaneous assessment of cytogenetic aberrations and immunophenotypic features of individual cells. S-100 protein and desmin antibodies were used to evaluate the chondrosarcomatous and rhabdomyosarcomatous components, respectively. A chromosome 7-specific centromeric probe was used for determination of aneuploidy. In both specimens obtained from the primary lesion, S-100 protein and desmin-positive and -negative aneuploid cells were observed. These findings: 1) suggest that both the chondrocytic and rhabdomyoblastic cells arose from the same abnormal clone, 2) support the theory of a common primitive mesenchymal cell progenitor with the ability to differentiate or express features of more than one line of mesenchymal differentiation, and 3) indicate that the term dedifferentiated may be an inaccurate designation for this neoplasm.
对一例具有横纹肌肉瘤成分的去分化软骨肉瘤的四个标本（活检标本、根治性手术标本以及两个分别出现的肺转移灶）进行细胞遗传学分析，结果显示每个标本均存在克隆性核型异常。所有标本中均可见的异常包括一条结构异常的17号染色体以及5、7、12和20号染色体的额外拷贝。通过一种联合免疫细胞化学/细胞遗传学方法确定染色体异常细胞的来源，该方法能够同时评估单个细胞的细胞遗传学畸变和免疫表型特征。分别使用S-100蛋白和结蛋白抗体来评估软骨肉瘤成分和横纹肌肉瘤成分。使用7号染色体特异性着丝粒探针来确定非整倍体情况。在取自原发性病变的两个标本中，均观察到S-100蛋白阳性和阴性以及结蛋白阳性和阴性的非整倍体细胞。这些发现：1）提示软骨细胞和成横纹肌细胞均起源于同一个异常克隆；2）支持存在一种具有分化能力或表达多种间充质分化谱系特征的共同原始间充质细胞祖细胞的理论；3）表明“去分化”一词可能并非该肿瘤的准确命名。
