Gastric leiomyosarcoma manifesting peculiar findings: Radiological-pathological correlation
Gastric leiomyosarcoma manifesting peculiar findings: Radiological-pathological correlation
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DOI：
10.1111/j.1440-1827.2009.02370.x
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发表时间：
2009-05-01
影响因子：
2.2
通讯作者：
Yanagisawa, Akio
中科院分区：
文献类型：
--
作者：
Masuzawa, Naoko;Kishimoto, Mitsuo;Yanagisawa, Akio
本文报告一例胃平滑肌肉瘤，现今极为罕见。人们不仅关注病理表现，还关注磁共振成像（MRI）表现的组织学基础。患者为一名29岁的日本女性。术前t2加权MRI显示大的高强度胃肿瘤伴等强度条纹。肿瘤为基础广泛的大息肉样病变，组织学上由梭形细胞束组成，胞浆嗜酸性，细胞核呈雪茄状。肿瘤细胞上的几种平滑肌标记物（包括desmin）的免疫反应性，聚合酶链反应中c-kit和PDGFRA cDNA的低扩增，以及外显子9和11中没有c-kit基因突变，强烈提示该肿瘤不是胃肠道间质瘤，而是真正的平滑肌肉瘤。新鲜肿瘤的体外MRI在形态学基础上解释放射学表现。体外MRI清晰地描绘了由放射状延伸的等强度线分隔的非常高强度的区域。这一影像学表现最符合最特征性的组织学特征，即肿瘤细胞呈线性延伸的束状，常伴有黏液性水肿改变，由呈放射状拉长的薄纤维血管间质隔开，即喷射性外观。
Reported herein is a gastric leiomyosarcoma, which, nowadays, is extremely rare. Attention was focused not only on pathological findings but also on the histological basis of magnetic resonance imaging (MRI) findings. The patient was a 29-year-old Japanese woman. Preoperative T2-weighted MRI showed a large high-intensity gastric tumor with isointense streaks. The tumor was a broad-based large polypoid lesion and histologically consisted of fascicles of spindled cells having eosinophilic cytoplasm and cigar-shaped nuclei. Immunoreactivity for several smooth muscle markers including desmin on tumor cells, low amplification of both c-kit and PDGFRA cDNA on polymerase chain reaction, and absence of c-kit gene mutation in exons 9 and 11 strongly suggested that the tumor was not a gastrointestinal stromal tumor but a true leiomyosarcoma. In vitro MRI of the fresh tumor was obtained to explain the radiological findings on a morphological basis. In vitro MRI clearly depicted the very high-intensity areas separated by radially extended isointense lines. This radiological finding corresponded best to the most characteristic histological feature, that is, linearly extended fascicles of the tumor cells often with myxedematous change separated by radially elongated thin fibrovascular stroma: in other words, spouting appearance.