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骨外间叶性软骨肉瘤

Extraskeletal mesenchymal chondrosarcoma.

作者信息

Shapeero L G, Vanel D, Couanet D, Contesso G, Ackerman L V

机构信息

Department of Radiology, Institut Gustave-Roussy, Villejuif, France.

出版信息

Radiology. 1993 Mar;186(3):819-26. doi: 10.1148/radiology.186.3.8430193.

Abstract

Among seven patients with extraskeletal mesenchymal chondrosarcoma (EMC), three children (aged 3-6 years) developed EMC in a central location and four adults (aged 38-54 years) developed EMC in both central and peripheral sites. Conventional radiography and tomography and computed tomography (CT) depicted EMC as a soft-tissue mass with ring, arc, stippled, and highly opaque calcifications in four patients. Contrast-enhanced CT showed lobulation and peripheral tumoral enhancement, sometimes with central low-attenuation areas. On magnetic resonance (MR) images, EMC was a lobulated mass with high signal intensity on T2-weighted images and enhancement with low-signal-intensity focal areas on contrast-enhanced T1-weighted images. All adults developed recurrences and/or metastases and died. Of the three children, two were living and free of disease at the end of the study and the third child died of chemotherapeutic-induced leukemia. Although imaging features of EMC are nonspecific, its chondroid-type calcifications and foci of low signal intensity within enhancing lobules may reflect its dual histopathologic morphologic characteristics of differentiated cartilage islands interspersed within vascular undifferentiated mesenchyme.

摘要

在7例骨外间叶性软骨肉瘤(EMC)患者中,3名儿童(3 - 6岁)的EMC发生于中央部位,4名成人(38 - 54岁)的EMC发生于中央和外周部位。传统X线摄影、体层摄影及计算机断层扫描(CT)显示,4例患者的EMC表现为软组织肿块,伴有环状、弧形、点状及高度致密的钙化。增强CT显示肿块呈分叶状,周边肿瘤强化,有时中央有低密度区。在磁共振(MR)图像上,EMC为分叶状肿块,在T2加权图像上呈高信号强度,在增强T1加权图像上有低信号强度的局灶性强化区。所有成人患者均出现复发和/或转移并死亡。3名儿童中,2名在研究结束时存活且无疾病,第3名儿童死于化疗诱导的白血病。尽管EMC的影像学特征不具有特异性,但其软骨样钙化及强化小叶内的低信号灶可能反映了其双重组织病理学形态特征,即分化的软骨岛散布于血管丰富的未分化间充质中。

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