The clinical characteristics of intra-abdominal aggressive fibromatosis

HUANG Yuanjian; XU Ziwei

doi:10.3969/j.issn.1674-4136.2025.01.013

Abstract：Objective To summarize and analyze the common clinical characteristics of patients with intra-abdominal aggressive fibromatosis(IAF),in order to enhance the understanding,diagnostic,and therapeutic level of this disease.Methods Establish a retrospective single-arm cohort,including 32 IAF patients who underwent surgery at the First Affiliated Hospital of Nanjing Medical University from 2017 to 2023.A summary analysis was conducted on their general conditions,imaging characteristics,surgical conditions,pathological features,and prognostics.Results The median age of the 32 IAF patients was 43 years,with a male predominance(56.3％).The most common initial symptom was abdominal pain(50.0％),and many had a history of gastric cancer surgery(18.8％).Solitary tumors were the most frequent(81.2％),typically located in the mid-abdomen involving the small intestine and its mesentery(56.2％).Tumor sizes varied from less than 5 cm(46.9％)to over 20 cm(3.1％).On imaging,tumors often appeared round-like with unclear boundaries from surrounding tissues.On CT scans,tumors predominantly showed slightly low density,while MRI demonstrated isointense or slightly low signals on T1WI and slightly high signals on T2WI.Both CT and MRI enhanced scans revealed heterogeneous enhancement.Most patients achieved R0 resection(90.6％).Postoperative pathological diagnosis relied on immunohistochemical staining including β-catenin,Vimentin,SMA,S-100,and CD117.The overall recurrence rate after R0 resection was 8％.Conclusions IAF is a rare tumor whose diagnosis depends on a detailed medical history,imaging,and immunohistochemical pathology examination.Comprehensive evaluation should be performed for IAF patients to select the optimal treatment strategy,thereby improving patient outcomes.