| 王宁,郑浩,朱泽斌,等.腹型侵袭性纤维瘤病 7例临床分析[J].安徽医药,2021,25(5):920-922. |
| 腹型侵袭性纤维瘤病 7例临床分析 |
| Clinical analysis of 7 cases of abdominal aggressive fibromatosis |
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| DOI：10.3969/j.issn.1009-6469.2021.05.018 |
| 中文关键词: 纤维瘤病，侵袭型 β-连环蛋白 病理特征 诊断与治疗 |
| 英文关键词: Fibromatosis, aggressive β-Catenin Pathological feature Diagnosis and treatment |
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| 中文摘要: |
| 目的探讨腹型侵袭性纤维瘤病( AF)的临床病理特征及诊疗。方法回顾性分析中国科学技术大学附属第一医院(安徽省立医院) 2015年 5月至 2019年 11月收治的 7例腹型侵袭性纤维瘤病的临床病理及随访资料。结果 7例病例中腹壁型侵袭性纤维瘤病 3例，腹内型侵袭性纤维瘤病 4例；既往史:腹壁型 1例腹部术史， 2例妊娠史；腹内型 4例均有腹部术史； 7例均无家族性肠腺瘤性息肉病史；腹壁型均以无症状肿块为首发症状，腹内型 3例以器官压迫症状为首发症状， 1例腹痛待查；腹壁型肿瘤部位均位于下腹壁，腹内型 3例来源于肠系膜， 1例位于盆腔侵犯膀胱及乙状结肠；腹壁型均行手术切除，腹内型 3例根治性手术切除， 1例开腹活检；免疫组织化学: β-连环蛋白( β-Catenin)均阳性， Dog-1检测 4例均阴性。术后中位随访 28.8月， 1例术后 1月行多学科讨论， 1例因联系方式变更失访， 1例术后 7月复发，无死亡病例。结论腹型侵袭性纤维瘤既往多有腹部术史，免疫组化 β-Catenin阳性和 Dog-1阴性有助于病理诊断，手术根治性切除是肠系膜型侵袭性纤维瘤病的首选治疗方式，该病总体预后良好。 |
| 英文摘要: |
| Objective To investigate the clinicopathological characteristics,, diagnosis and treatment of abdominal aggressive fibromatosis (AF).Methods The clinicopathological and follow-up data of 7 cases of AF admitted to the First Affiliated Hospital of the University of Science and Technology of China (Anhui Provincial Hospital) from May 2015 to November 2019 were retrospectively analyzed.Results Among the 7 patients,there were 3 cases of abdominal wall AF and 4 cases of intra-abdominal AF. Past medical history:1 case of abdominal wall AF had abdominal surgery history, 2 cases had a history of pregnancy; 4 cases of intra-abdominal AF had a history of abdominal surgery; All cases had no history of familial adenomatous polyposis; All patients of abdominal wall AF were presented with asymptomatic mass. Among the intra-abdominal AF, 3 patients presented with organ compression symptoms, 1 case presented with abdominal pain; All of abdominal wall AF located in the low abdominal wall. Among the intra-abdominal AF, 3 cases located onthe mesentery, 1 case in pelvic cavity; 6 patients underwent extensive tumor resection include 3 abdominal wall cases and 3 intra-abdominal cases. The pelvic patient received exploratory biopsy because of infiltrated urinary bladder and sigmoid colon. Immunohistochemistry: β-Catenin test was positive for all patients; Dog-1 test was negative in 4 cases. The median follow-up was 28.8 months afteroperation. One patient underwent multidisciplinary discussion at 1 month after surgery, one patient was lost to follow-up due to changes in contact information, and one patient relapsed 7 months after operation. All cases had no deaths occurred.Conclusions AF always has a history of abdominal surgery, β-Catenin positive and Dog-1 negative of pathology,these contribute to final diagnosis. Surgical radical resection is the preferred treatment of mesenteric AF. the overall prognosis is well |
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