CAJ | 학술논문

目的：探讨胰腺胃肠外恶性间质瘤（ EGIST）的临床特点、诊断及治疗。方法：分析1例胰腺 EGIST 患者的诊治情况，并复习有关文献。结果：患者，男，53岁，胰腺肿瘤大小30.0cm ×25.0cm ×20.0cm，与周围组织广泛粘连。在组织学上，肿瘤细胞呈栅栏状或短束状交织排列，伴上皮样细胞分化，核分裂象3-5个/10HPF。鉴别诊断包括胰腺平滑肌瘤和神经内分泌肿瘤。免疫组织化学显示肿瘤细胞表达CD117、CD34、DOG-1、CD99、Bcl-2和vimentin，不表达SMA、S-100、Desmin、CD31、Cytokeratin、NSE、CgA和Syn。患者行胰腺肿瘤切除术，诊断为胰腺EGIST。结论：胰腺EGIST临床罕见，无特征性临床表现，以手术切除治疗为主，转移和不能手术治疗者可采取分子靶向治疗。
목적：탐토이선위장외악성간질류（ EGIST）적림상특점、진단급치료。방법：분석1례이선 EGIST 환자적진치정황，병복습유관문헌。결과：환자，남，53세，이선종류대소30.0cm ×25.0cm ×20.0cm，여주위조직엄범점련。재조직학상，종류세포정책란상혹단속상교직배렬，반상피양세포분화，핵분렬상3-5개/10HPF。감별진단포괄이선평활기류화신경내분비종류。면역조직화학현시종류세포표체CD117、CD34、DOG-1、CD99、Bcl-2화vimentin，불표체SMA、S-100、Desmin、CD31、Cytokeratin、NSE、CgA화Syn。환자행이선종류절제술，진단위이선EGIST。결론：이선EGIST림상한견，무특정성림상표현，이수술절제치료위주，전이화불능수술치료자가채취분자파향치료。
Objective:To study the clinical manifestation,diagnosis and treatment of extra-gastrointestinal stro-mal tumor( EGIST)in pancreas. Methods:One case of extra gastrointestinal stromal tumor in pancreas was presented with review of the literature. Results:We described a 53-year-old man with a malignant pancreatic gastrointestinal stromal tumor. The tumor which extensive adhesion with the surrounding organs,was measured 30. 0cm × 25. 0cm × 20. 0cm. On histology,it showed a mixed spindle and epithelioid cell morphology with the presence of sheets and short intersecting fascicles of tumor cells. The mitotic count was 3-5 mitoses per 10 high-power fields. The differential di-agnosis included pancreatic smooth muscle tumor and neuroendocrine tumor. Immunohistochemistry revealed diffuse cytoplasmic positivity for CD117,CD34,DOG-1,CD99,Bcl-2 and vimentin. Tumor cells were negative for SMA,S-100,desmin,CD31,cytokeratin,NSE,chromogranin and synaptophysin. Conclusion:Pancreatic extra-gastrointes-tinal stromal tumor is extremely rare and there were only seven previous reports in the literature. Without particular clinical features,the main therapy is surgery. Unresectable and metastatic cases can be treated with molecular targeted therapy.