摘要
目的分析肺外发生的炎症性肌纤维母细胞瘤(IMT)的临床病理特点、免疫组化及生物学行为。方法对10例肺外IMT进行临床和病理资料分析、免疫组化检测及随访。结果本组10例IMT,男、女各5例;发病年龄为13～69岁,中位年龄40岁。发生部位包括胃、结肠、四肢及头面部软组织、肾上腺。临床主要表现为局部肿块及由其引起的压迫症状。组织学主要表现为梭形纤维母细胞与肌纤维母细胞混杂排列,间质见含有浆细胞的炎细胞浸润,可见不同程度的玻璃样变及黏液疏松背景。免疫组化:肿瘤细胞vimentin均(+)、大部分SMA(+);部分desmin和ALK-1(+),CD117(-)。随访9例,8例无瘤生存,1例肿瘤复发并恶变。结论肺外IMT是少见的良性肿瘤,但少数具有复发倾向及恶变潜能,故定期随访非常重要。该病需与梭形细胞肿瘤进行鉴别。
Objective To investigate the clinicopathologieal characteristics, immunophenotypes and clinical prognosis of inflammatory myofibroblastic tumor （IMT） of extrapulmonary tissue. Methods The clinicopathologie features of ten cases of the IMT of extrapulmonary tissue were analysed, immunohistochemical staining and follow-up were performed. Results The age of the ten cases was from 13 to 69 years, with the mean of 40 years, and the ratio of female to male was 1 ： 1. The primary sites of IMT of extrapulmonary tissue included stomach, colon, soft tissue of the limbs, head and face, and adrenal gland. The main clinical symptoms developed from the compression of a local mass. Histologically, the tumor was characterisized by mixed arrangement of spindle-cells, fibroblast and myofibroblast, infiltration of inflammatory cells including plasmocytes in the stroma, varying degrees of hyaline degeneration and myxiod back;ground. Almost all cases of IMT of extrapulmonary tissue expressed vimentin and SMA, some cases expressed desmin, ALK-1, but none case expressed CD117. Follow-up data showed that one male patient recurred locally after 2 years with malignant transformation, and other cases had no sign of local recurrence and metastasis. Conclusion Most of IMT of extrapulmznary tissue are rare benign tumors, a few cases have malignant potential, and therefore close follow-up after operation is very important. The diagnosis of the IMT of extrapulmonary tissue depends on histopathologieal changes and immunohistochemical results. More attention should he paid to differentiate it from the tumors of spindle cell similar to IMT.
出处
《诊断病理学杂志》
CSCD
2010年第5期325-327,共3页
Chinese Journal of Diagnostic Pathology
关键词
炎症性肌纤维母细胞瘤
临床病理
免疫组化
肺外组织
Inflammatory myofibroblastic tumor
Clinicopathology
Immunohistochemistry
Extrapulmonary tissue
