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累及消化道的腹内型侵袭性纤维瘤14例临床分析

Clinical analysis of 14 patients with intraperitoneal desmoid tumor fibromatosis invading the digestive tract
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摘要 目的探讨累及消化道的腹内型侵袭性纤维瘤(DTF)患者的临床、影像学及病理学特点。方法选择2003年1月至2021年1月郑州大学第一附属医院收治的14例累及消化道的腹内型DTF患者为研究对象,回顾性分析其临床特点、影像学表现、组织病理学特点。结果CT检查显示,9例患者可见软组织肿块,增强后呈轻中度强化,病变与消化道关系密切。超声检查显示,8例患者腹部中低回声实性肿块,边界不清晰,回声不均匀。胃镜检查显示,1例患者胃窦部可见黏膜下隆起,黏膜表面光滑。14例患者术前穿刺(4例)或术后切除标本(10例)病理学检查均诊断为DTF,苏木精-伊红(HE)染色均提示为梭形细胞肿瘤。13例(12例外科手术切除,1例胃镜下切除)患者病理切片HE染色均未见明显包膜,大体表现为灰白色、质硬包块。14例患者DTF组织中β-catenin、平滑肌肌动蛋白(SMA)、Desmin蛋白阳性表达分别为9例(64.29%)、8例(57.14%)、3例(21.43%)。14例患者平均随访时间7~72(56.8±17.0)个月,生存率为100%(14/14),局部复发2例(14.3%),均无远处转移。结论累及消化道的腹内型DTF临床较为罕见,为梭形细胞肿瘤,无明显包膜,DTF组织中β-catenin、SMA蛋白表达阳性率较高,手术切除仍是该型DTF重要的治疗手段,但复发率较高。 Objective To investigate the clinical,imaging and pathological characteristics of patients with intraperitoneal desmoid tumor fibromatosis(DTF)invading the digestive tract.Methods A total of 14 patients with intraperitoneal DTF invading the digestive tract admitted to the First Affiliated Hospital of Zhengzhou University from January 2003 to January 2021 were selected as the research objects,and their clinical features,imaging manifestations,histopathological features were retrospectively analyzed.Results CT examination showed that soft tissue mass was seen in 9 cases,which showed mild to moderate enhancement after enhancement,and the lesions were closely related to the digestive tract.Ultrasound examination showed that 8 cases of low-echoic solid mass in abdomen with unclear boundary and uneven echo.Gastroscopy showed that submucosal bulge was seen in the gastric antrum in 1 case,and the surface mucosa was smooth.DTF was diagnosed by pathological examination of preoperative puncture(4 cases)or postoperative resection specimens(10 cases)of 14 patients,and hematoxylin-eosin(HE)staining indicated spindle cell tumor.Thirteen cases(12 cases of surgical resection,1 case of gastroscopic resection)had no obvious capsule in the pathological sections of the patients,and the general appearance was gray-white and hard mass.The positive expressions ofβ-catenin,smooth muscle actin(SMA)and Desmin protein in DTF tissues of 14 patients were 9 cases(64.29%),8 cases(57.14%)and 3 cases(21.43%),respectively.The average follow-up time of 14 patients was 7-72(56.8±17.0)months,the survival rate was 100%(14/14),and 2 patients(14.3%)had local recurrence,and none of them had distant metastasis.Conclusion Intraperitoneal DTF invading the digestive tract is relatively rare,it is a spindle cell tumor without obvious capsule,the positive rate ofβ-catenin and SMA protein expression in DTF tissue is high,and surgical resection is still an important treatment method for this type DTF,but the recurrence rate is high.
作者 郑庆芬 刘冰熔 周洋洋 刘丹 ZHENG Qingfen;LIU Bingrong;ZHOU Yangyang;LIU Dan(Department of Gastroenterology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,Henan Province,China)
出处 《新乡医学院学报》 CAS 2022年第6期562-565,共4页 Journal of Xinxiang Medical University
关键词 消化道 腹内型侵袭性纤维瘤 临床分析 digestive tract intraperitoneal desmoid tumor fibromatosis clinical analysis
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