Kozu Yoshiki, Isaka Mitsuhiro, Ohde Yasuhisa, Takeuchi Kengo, Nakajima Takashi
Division of Thoracic Surgery, Shizuoka Cancer Center, Shimonagakubo 1007, Nagaizumi, Shizuoka, 411-8777, Japan,
Gen Thorac Cardiovasc Surg. 2014 Mar;62(3):191-4. doi: 10.1007/s11748-013-0204-x. Epub 2013 Jan 24.
A 57-year-old Japanese man presented with massive right pleural effusion, and a huge tumor arising in the pleural cavity was detected by chest computed tomography. A thoracoscopic tumor biopsy revealed that the tumor protruded extensively into the pleural cavity, and its gross appearance was cystic and glossy. Microscopically, the tumor cells were rounded and epithelioid in shape. Prominent and abundant myxoid stroma was also present together with an inflammatory infiltrate, and the tumor was anaplastic lymphoma kinase (ALK)-immunopositive. Fluorescence in situ hybridization revealed that the Ran-binding protein 2-ALK fusion gene was present. Taken together, these findings supported the diagnosis of epithelioid inflammatory myofibroblastic sarcoma (EIMS), which is a variant of an inflammatory myofibrobrastic tumor. This is the first reported case of an EIMS arising in the pleural cavity.
一名57岁的日本男性因大量右侧胸腔积液就诊，胸部计算机断层扫描发现胸腔内有一个巨大肿瘤。胸腔镜肿瘤活检显示，肿瘤广泛突入胸腔，肉眼外观呈囊性且有光泽。显微镜下，肿瘤细胞呈圆形且为上皮样。还存在显著且丰富的黏液样基质以及炎症浸润，肿瘤间变性淋巴瘤激酶（ALK）免疫阳性。荧光原位杂交显示存在Ran结合蛋白2-ALK融合基因。综合这些发现，支持上皮样炎性肌成纤维细胞肉瘤（EIMS）的诊断，EIMS是炎性肌成纤维细胞瘤的一种变体。这是首例报道的发生于胸腔的EIMS病例。
