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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。
Objective: To investigate the clinical pathology, immunohistochemistry and ultrastructure of intra-abdominal fibromatosis and discuss its differential diagnosis. Methods: Immunohistochemical and electron microscopic methods, combined with HE morphological observation, 17 cases of intra-abdominal fibromatosis were studied. Results: There were 7 males and 10 females, aged from 19 to 78 years old, with an average age of 429 years and a median age of 42 years. Four patients had a history of surgery and two had Gardner’s syndrome. 15 cases of single nodules, 2 cases of 2 nodules. Mass diameter of 5 ~ 2 5cm, an average of 13 5cm, most of the realm clear, gray-white tough section, solid, translucent. Light microscopy mainly slender spindle and star-shaped two forms of (muscle) fibroblasts, a large number of interstitial collagen. Ultrastructure showed a large number of collagen fibroblasts around proliferative cells, clear plasma membrane, no substrate, abundant intracellular rough endoplasmic reticulum. Immunophenotype: Vim diffuse strongly positive; 46.7% (7/15); α SMA focal positive; 40% (6/15) MSA focal positive; CD117, CD34, S 10 0 protein, PGP9 5, desmin negative. There were 15 cases with complete mass and partial resection, 7 without recurrence (2 ~ 13 years, mean 8 years), 3 lost follow-up and 5 with follow-up. Palliative surgery in 2 cases, 1 case died of intestinal obstruction, 1 case was lost after surgery. CONCLUSION: Intra-abdominal fibromatosis is a rare and misdiagnosed disease, partly related to the history of surgery and Gardner’s syndrome. The possible mechanisms are the imbalance of collagen synthesis and degradation, the characteristic of myofibroblastic differentiation by immunohistochemistry, the complete resection of the operation Good treatment effect.