摘要
目的 :探讨腹内纤维瘤病临床病理、免疫组化和超微结构特点 ,讨论其鉴别诊断。方法 :用免疫组化及电镜的方法 ,结合HE形态学观察 ,对 17例腹内纤维瘤病进行研究。结果 :男 7例 ,女 10例 ,年龄 19～ 78岁 ,平均 4 2 9岁 ,中位年龄 4 2岁。 4例有手术史 ,2例有Gardner综合征。 15例单结节 ,2例 2枚结节。肿块直径 5～ 2 5cm ,平均 13 5cm ,多数境界清楚、切面灰白质韧、实性、半透明状。光镜下主要有纤细梭长和星芒状两种形态的 (肌 )纤维母细胞 ,间质大量胶原。超微结构显示增生细胞周围大量胶原原纤维 ,质膜清楚 ,无基板 ,胞质内有丰富的粗面内质网。免疫表型 :Vim弥漫强阳性 ;4 6 7% (7/ 15 ) ;α SMA局灶阳性 ;4 0 % (6 / 15 )MSA局灶阳性 ;CD117、CD34、S 10 0蛋白、PGP9 5、desmin阴性。行完整肿块及部分肠段切除者 15例 ,7例无复发 (2～ 13年 ,平均 8年 ) ,3例失访 ,5例随访中。姑息术者 2例 ,1例死于肠梗阻 ,1例再次术后失访。结论 :腹内纤维瘤病是少见且易误诊的疾病 ,部分与手术史以及Gardner综合征有关 ,可能的机制为胶原合成和降解失衡 ,免疫组化显示部分肌纤维母细胞分化的特点 ,手术完整切除者治疗效果好。
Purpose To explore the clinicopathologic and electron microscopic features of intra abdominal fibromatosis(IAFs), and discuss the differential diagnosis. Methods IAFs were studied by immunohistochemic and electron microscopic methods. Results IAFs occurred in 7 men and 10 women with a mean age of 42 9 year(range, 19～78 years), four cases had previous abdominal surgery and two cases was associated with Gardner's syndrome. Fifteen cases showed a nodule and two, multinodules. The lesions were measured from 5 cm to 25 cm (mean 13 5 cm) in diameter. Most of them were demarcated. The cut surface was pale grey, whorled and pliable, with areas of gelatinous material and half transparent. Microscopically, the masses were composed of spindle and corpulent shaped cells, separated by a variable amount of collagenous tissue. Electron microscopically revealed abundant extracellular collagen with spindle cells buried throughout. The cells contained large amounts of rough endoplasmic reticulum, part of cells contained intracytoplasmic collagen. Immunohistochemically, all of them showed diffuse strong positive for vimentin, 7 of 15(46 7%) focal positive for α SMA and 6 of 15 (40%)also local positive for MSA,all were negative for desmin?CD117?CD34?S 100?PGP9 5 were negative. The mass with part of intestine were resected in 15 cases, seven cases were alive without recurrence after an average period of 8 years (range from 2 to 13 years), three were failed to follow up, five cases in follow up, two were incompletely excised, one was died of intestinal obstruction and the other was failed to follow up after second partial resection. Conclusions IAFs are a rare intestinal obstruction, some cases are associated with previous abodominal surgery and colonic polyposis and is then considered to be a component of Gardner's syndrome, the machanism might be unbalance of synthesis and decomposis of collegen. Immunohistochemical staining showed part differentiation of myofibroblasts. It's appeared to be favorable prognosis after complete resection.
出处
《临床与实验病理学杂志》
CAS
CSCD
2002年第2期127-132,共6页
Chinese Journal of Clinical and Experimental Pathology
基金
复旦大学三年行动计划 (985工程 NoEXF15 93 0 1)
