腹内型韧带样型纤维瘤病的CT表现(附4例报告并文献复习)

CT Manifestations of Intra-Abdominal Desmoid-Type Fibromatosis:Report of 4 Cases with Literature Review

目的探讨腹内型韧带样型纤维瘤病(DF)的CT表现。方法搜集4例经手术病理证实的腹内型DF的临床及影像资料,均行CT平扫及多期增强扫描,结合文献回顾性分析其CT特征。结果 4例均为单发病变,分别位于小肠系膜、回结肠系膜、大弯侧胃壁、肝脏;均呈类圆形或分叶状软组织肿块,轮廓清晰,体积较大,最大径线8.0~15.0 cm,平均10.3 cm;平扫时3例相对骨骼肌呈均匀低密度,增强扫描呈渐进性、不均质、轻-中度强化;1例相对肝实质呈混杂略低密度,内见斑片状更低密度区,动脉期片絮状明显强化,门静脉期及延迟扫描呈持续强化,强化范围逐步扩大,强化区密度均匀,夹杂有裂隙状低密度区。结论腹内型DF的CT表现具有一定的特征性,可提示诊断,确诊仍需病理及免疫组织化学证实。

Objective To investigate the CT imaging features of intra-abdominal desmoid-type fibromatosis and to improve the understanding of this disease. Methods CT imaging features of intra-abdominal desmoid-type fibromatosis in four patients confirmed by postoperative histopathology were reviewed retrospectively. Moreover,the related literature was reviewed. Results Tumors were found in the small bowel mesentery,ileocolic mesentery,stomach and liver,respectively. All lesions displayed larger well-circumscribed,expansive,ovate or lobulated solid mass,ranging from 8. 0 cm to 15. 0cm in greatest diameter with a mean of 10. 3 cm. Three lesions showed homogeneous hypodense attenuation with respect to skeletal muscle on unenhanced CT,gradually heterogeneous and modest enhancement on contrast-enhanced CT. The lesion in the liver appeared heterogeneous hypodensity and showed a fast and intense heterogeneous enhancement in arterial phase,and gradual and prolonged enhancement in portal venous and delayed phases. Conclusion The CT imaging features of intra-abdominal desmoid-type fibromatosis may be helpful for the diagnosis,nevertheless,the definitive diagnosis is established by pathological and immunohistochemical features.