摘要
9例肠系膜韧带样纤维瘤病患者于2010年1月至2022年5月就诊于温州医科大学附属台州医院,其中男性7例,女性2例,年龄16~59岁;肿瘤位于小肠系膜7例,回盲部系膜1例,横结肠系膜1例;肿瘤最大径3.5~33.0 cm,平均(10.7±8.5)cm,无包膜,界不清,部分病例侵犯肠肌壁组织,切面呈实性,灰白色,质韧。组织病理学检查见梭形的纤维母细胞及肌纤维母细胞平行状、束状或交错排列,埋于大量细胞外胶原内,细胞形态较一致,无明显异型性,核分裂象少见。免疫组织化学检测结果显示,9例患者的肿瘤细胞波形蛋白、β联蛋白均阳性,5例患者α-平滑肌肌动蛋白阳性,Ki-67阳性指数为1%~10%,广谱细胞角蛋白、S-100蛋白、信号转导及转录激活蛋白6、CD117、DOG1、CD34、结蛋白和间变性淋巴瘤激酶均阴性。7例存在CTNNB1基因c.121G>A(p.Thr41Ala)突变,1例c.121G>A(p.Thr41Ala)和c.134C>T(p.Ser45Phe)双突变,1例为野生型。9例患者均接受手术切除,8例无复发及转移;1例半年后复发,再次手术切除后无复发及转移。
Nine cases of mesenteric desmoid-type fibromatosis were diagnosed and treated in Taizhou Hospital,Wenzhou Medical University between January 2010 and May 2022,including 2 females and 7 males,aged 16 to 59 years.The lesions were in the mesentery of small intestine with 7 cases,ileocecal junction with 1 cases and transverse colon with 1 case. The tumors had an unclear boundary and no envelope, the section was solid, gray and tough. The mean maximum diameter was (10.7±8.5) cm (range 3.5-33.0 cm). Microscopically, fusiform fibroblasts and myofibroblasts were parallel, bunched or staggered, buried in a large amount of extracellular collagen. The cell morphology was relatively consistent, without obvious atypia, and mitosis was rare. Immunohistochemistry showed that the tumor cells were positive for vimentin (9/9), β-catenin (9/9), while smooth muscle actin (5/9) stains were focally positive. Ki-67 proliferation index was 1%-10%. Cytokeratin Pan, S-100, STAT6, CD117, DOG1, CD34, desmin and anaplastic lymphoma kinase stains were negative. Genetic analysis showed that there were 7 cases of c.121G>A(p.Thr41Ala) mutation of CTNNB1 gene, 1 case of c. 121G>A(p. Thr41Ala) and 1 case of c. 134C>T(p. Ser45Phe) double mutation, and 1 case of wild type. Tumors were surgically resected in all 9 cases. Eight cases had no recurrence or metastasis, 1 case had recurrence 6 months later, and no recurrence or metastasis after additional surgical resection.
作者
王秋鹏
张玲娜
翁寿向
周晶晶
甘梅富
WANG Qiupeng;ZHANG Lingna;WENG Shouxiang;ZHOU Jingjing;GAN Meifu(Department of Pathology,Taizhou Hospital,Wenzhou Medical University,Taizhou 317000,Zhejiang Province,China)
出处
《浙江大学学报（医学版）》
CAS
CSCD
北大核心
2023年第3期379-385,共7页
Journal of Zhejiang University(Medical Sciences)
