Lee Yoon Young, Kim Do Hoon, Lee Ji Hye, Choi Jong Sang, In Kwang Ho, Oh Yu Whan, Cho Kyung Hwan, Roh Yong Kyun
Department of Family Medicine, Korea University, College of Medicine, Seoul, Korea.
J Korean Med Sci. 2007 Sep;22 Suppl(Suppl):S159-63. doi: 10.3346/jkms.2007.22.S.S159.
Extraskeletal Ewing's sarcoma (EES) is a branch of neuroectodermal tumor (PNET), which is very rare soft tissue sarcoma. We report a case of EES/PNET arising is the lung of a 67-yr-old man. Computed tomography, bone scintigraphy, and positron emission tomography confirmed the mass to have a primary pulmonary origin. The mass showed positive reactivity in the Periodic Acid Schiff (PAS) stain and MIC-2 immunoreactivity in immunohistochemical stain. Fluorescence in situ hybridization (FISH) was performed, which revealed an EWSR1 (Ewing sarcoma breakpoint region 1) 22q12 rearrangement. The diagnosis was confirmed both pathologically and genetically. The mass lesion was resected, and the patient is currently undergoing chemotherapy.
骨外尤文肉瘤(EES)是神经外胚层肿瘤(PNET)的一个分支,是一种非常罕见的软组织肉瘤。我们报告一例发生于一名67岁男性肺部的EES/PNET病例。计算机断层扫描、骨闪烁显像和正电子发射断层扫描证实该肿块起源于肺部。该肿块在高碘酸希夫(PAS)染色中呈阳性反应,在免疫组织化学染色中呈MIC-2免疫反应性。进行了荧光原位杂交(FISH),结果显示存在EWSR1(尤文肉瘤断点区域1)22q12重排。病理和基因诊断均得到证实。切除了肿块病变,患者目前正在接受化疗。