Clinicopathological features of extraskeletal myxoid chondrosarcoma: An analysis of 9 cases
Clinicopathological features of extraskeletal myxoid chondrosarcoma: An analysis of 9 cases
复制标题
骨外粘液样软骨肉瘤9例临床病理特征分析
DOI：
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发表时间：
2008
期刊：
影响因子：
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通讯作者：
Qiong Liu
中科院分区：
文献类型：
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作者：
Xiufen Ye;C. Mi;Yu Li;Qiong Liu
目的探讨皮肤外粘液样软骨肉瘤（EMC）的临床病理特点。对所有病例进行了广泛的免疫组化分析，并对2例皮肤外粘液样软骨肉瘤进行了超微结构研究。随访资料为7 patients.ResultsThere是7名男性和2名女性，年龄范围从31至69岁（中位数52.78岁）。局部疼痛或触痛以及存在可触及的肿块是患者的主要抱怨。肿瘤好发于下肢（66.7%）。大多数肿瘤位于深部。它们通常具有明显的多结节结构，由纤维结缔组织界定。肿瘤细胞大部分呈纤细的交叉股、环和花环状排列。多数病例粘液样基质丰富。所有病例均行免疫组化染色，2例皮肤外粘液样软骨肉瘤行超微结构观察。EMC表达波形蛋白（100%，9/9）、神经元特异性烯醇化酶（77.8%，7/9）、S-100蛋白（66.7%，6/9）、突触素和嗜铬粒蛋白A（22.2%，2/9）。EMA和结蛋白均不表达。超微结构：EMC的特征是浸在富含糖胺聚糖的基质中的明显的细胞索。细胞线粒体丰富，高尔基体发达，有许多光滑的小泡。在许多细胞中，也有明显的糖原沉积和脂滴。部分肿瘤细胞有脑池内微管。2例皮肤外粘液样软骨肉瘤中1例胞体内可见直径140-180 nm的膜结合致密核心分泌颗粒。结论皮肤外粘液样软骨肉瘤（EMC）是一种罕见的软组织肉瘤，具有独特的形态学和细胞遗传学特征。然而，软骨的性质一直是一个有争议的话题，其分化线仍有待确定。相当大比例的EMC显示神经内分泌分化的免疫表型和/或超微结构证据。EMC有很高的局部复发和转移的可能性，以及很高的疾病相关死亡率。
ObjectiveTo investigate the Clinicopathological characteristics of extraskeletal myxoid chondrosarcoma (EMC).MethodsNine cases of extraskeletal myxoid chondrosarcoma were studied. Extensive immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas. Follow-up information was available for seven patients.ResultsThere were 7 males and 2 females whose ages ranged from 31 to 69 years (median 52.78 years). Local pain or tenderness and the presence of a palpable mass were the main complaints of the patients. The tumors were located mainly in the lower extremities (66.7%). Most tumors were deep-seated. They usually had a distinct multinodular configuration delineated by fibrous connective tissue. The tumor cells were arranged in delicate intersecting strands, rings, and garlands for the most part. The myxoid matrix was abundant in most cases. Immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas. EMC expressed vimentin (100%, 9/9), neuron-specific enolase (77.8%, 7/9), S-100 protein (66.7%, 6/9), synaptophysin and chromogranin A (22.2%, 2/9). None of the tumors expressed EMA and desmin. Ultrastructurally: EMC was characterized by distinct cords of cells immersed in a glycosaminoglycan rich matrix. The cells were rich in mitochondria, had well-developed Golgi apparatus and there were numerous smooth vesicles. In many cells, there were also prominent glycogen deposits and lipid droplets. Some tumor cells had intracisternal microtubules. In one of the 2 extraskeletal myxoid chondrosarcomas there were 140–180 nm diameter membrane-bound dense-core secretory granules in cell bodies.ConclusionExtraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma characterized by distinctive morphological and cytogenetical features. However, the chondroid nature has been a subject of controversy, and its line of differentiation remains to be determined. A substantial proportion of EMC shows immunophenotypic and/or ultrastructural evidence of neuroendocrine differentiation. EMC has high potential of local recurrence and metastasis, and a high disease-associated death rate.
EWS 与骨外粘液样软骨肉瘤孤儿核受体基因融合的分子分析。
DOI：
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发表时间：
1997
期刊：
The American journal of pathology
影响因子：
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作者：
Brody,RI;Ueda,T;Hamelin,A;Jhanwar,SC;Bridge,JA;Healey,JH;Huvos,AG;Gerald,WL;Ladanyi,M
通讯作者：
Ladanyi,M