腹壁侵袭性纤维瘤病1例并文献复习

A case of aggressive fibromatosis of the abdominal wall and literature review

目的 探讨侵袭性纤维瘤病的临床特点、影像学特征、病理学表现、鉴别诊断、治疗及预后,为该病的临床诊治提供参考。方法 报告一例术前被误诊为腹壁瘢痕子宫内膜异位症的腹壁侵袭性纤维瘤病,并复习相关文献。结果 侵袭性纤维瘤病是一种临床罕见的、具有局部侵袭性的交界性肿瘤,由成纤维细胞异常增生所致,好发于腹壁、肌层和筋膜鞘。其影像学检查无特异性,诊断与鉴别诊断主要依靠病理学检查,目前其治疗方式以手术治疗为主,其他方式包括放疗、化疗、内分泌治疗及靶向治疗等。结论 侵袭性纤维瘤病是一种发病率低、侵袭性强、复发率高、发病机制不明确的交界性肿瘤。应结合其临床表现、影像学及病理学特点诊断本病,应根据患者的情况制定个性化治疗措施。

Objective To investigate the clinical features,imaging features,pathological features,differential diagnosis,treatment and prognosis of aggressive fibromatosis,and to provide reference for clinical diagnosis and treatment of this disease.Methods Report a case of aggressive fibromatosis of the abdominal wall that was misdiagnosed as abdominal wall scar endometriosis before operation,and review relevant literature.Results Aggressive fibromatosis is a clinically rare,locally aggressive borderline tumor caused by the abnormal proliferation of fibroblasts,mainly occurring in the abdominal wall,muscular layer and fascial sheath.Its imaging examination has no specificity,and its diagnosis and differential diagnosis mainly rely on pathological examination.At present,surgical treatment is the main treatment,other methods include radiotherapy,chemotherapy,endocrine therapy and targeted therapy.Conclusion Aggressive fibromatosis is a borderline tumor with low incidence,strong invasiveness,high recurrence rate and unclear pathogenesis.The diagnosis of this disease should be combined with its clinical manifestations,imaging and pathological features,and individualized treatment measures should be formulated according to the patient’s situation.