CAJ | 학술논문

目的：探讨腹腔上皮样炎性肌纤维母细胞肉瘤( epithelioid inflammatory myofibroblastic sarcoma, EIMS)的临床病理学特征、诊断和鉴别诊断、分子遗传学进展、治疗及预后。方法回顾性分析2例EIMS的临床表现、大体及组织学形态、免疫表型,并复习相关文献。结果例1,男性,15岁；例2,女性,21岁,均因腹部疼痛不适入院,入院后行肿物切除术。肿瘤由致密区和富于黏液的疏松区构成,肿瘤细胞圆形、上皮样,核圆,核仁明显,可见病理性核分裂及瘤巨细胞,伴肿瘤性坏死,背景中可见丰富的炎细胞,以中性粒细胞为主体伴少量淋巴细胞和浆细胞。免疫表型：肿瘤细胞表达ALK、vimentin、desmin和CK (AE1/AE3)(灶阳性),不表达Calretinin、CD30、CD31、CD33、SMA、HHF35、Myogenin、S-100、HMB-45、CD20、CD79a、CD3、CD5、CD45和CD68。 FISH检测显示2例均有ALK基因相关易位。结论腹腔EIMS罕见,作为炎性肌纤维母细胞肿瘤的独立亚型之一,其正确诊断尤为重要。 ALK抑制剂或使ALK阳性的EIMS患者获益。
목적：탐토복강상피양염성기섬유모세포육류( epithelioid inflammatory myofibroblastic sarcoma, EIMS)적림상병이학특정、진단화감별진단、분자유전학진전、치료급예후。방법회고성분석2례EIMS적림상표현、대체급조직학형태、면역표형,병복습상관문헌。결과례1,남성,15세；례2,녀성,21세,균인복부동통불괄입원,입원후행종물절제술。종류유치밀구화부우점액적소송구구성,종류세포원형、상피양,핵원,핵인명현,가견병이성핵분렬급류거세포,반종류성배사,배경중가견봉부적염세포,이중성립세포위주체반소량림파세포화장세포。면역표형：종류세포표체ALK、vimentin、desmin화CK (AE1/AE3)(조양성),불표체Calretinin、CD30、CD31、CD33、SMA、HHF35、Myogenin、S-100、HMB-45、CD20、CD79a、CD3、CD5、CD45화CD68。 FISH검측현시2례균유ALK기인상관역위。결론복강EIMS한견,작위염성기섬유모세포종류적독립아형지일,기정학진단우위중요。 ALK억제제혹사ALK양성적EIMS환자획익。
Purpose To explore the clinicopathologic characteristics, immunophenotype, diagnosis and differential diagnosis, molecu-lar genetic feature, treatments and prognosis of intra-abdominal EIMS. Methods Two cases of intra-abdominal EIMS were studied with clinical manifestations, histology and immunohistochemical staining, and its clinical and pathological findings were further ana-lyzed with review of the literature. Results Case 1 was a 15-year-olds male and case 2 was a 21-year-olds female both of whom pres-ented with abdominal pain. Two patients were treated by surgical excision. Microscopically the tumor consisted of two different histolog-ical types, one of which was of high cell density and the other with low cell density and myxoid stroma. Both of these areas contained inflammatory cells, mainly neutrophils with few lymphocytes and plasmocytes. Tumor cells had an epithelioid phenotype with round nu-clei and small nucleoli, various nuclear atypia and mitotic figures were also found, which consistented with the diagnosis of epithelioid inflammatory myofibroblastic sarcoma. Immunohistochemical analysis revealed that the tumor cells were positive for ALK, vimentin, desmin, and CK(AE1/AE3) (focal), and were negative for Calretinin, CD30, CD31, CD33, SMA, HHF35, Myogenin, S-100, HMB-45, CD20, CD79a, CD3, CD5, CD45 and CD68. ALK rearrangement was identified in both cases by FISH using ALK break-a-part probe. Conclusions As an extremely rare tumor, the distinguishing between epithelioid inflammatory myofibroblastic sarcoma and conventional inflammatory myofibroblastic tumor is important. ALK inhibitors are theoretically useful for treating these tumors.