韧带样型纤维瘤病的影像表现及其病理基础

目的 探讨韧带样型纤维瘤（DF）的影像学特征及病理学基础.方法 回顾性分析经手术病理证实的韧带样型纤维瘤14例,6例行CT检查,其中 3 例增强检查;8例行 MRI 检查,其中7例增强.分析CT及MRI表现,并对肿瘤的病理特点进行分析.结果 肿瘤平均直径6.2cm.肿瘤形态为不规则形或分叶状11例（11/16）.肿瘤与周围结构分界不清或部分分界不清的9例（9/16）.7个肿瘤在CT上呈稍低或等密度,3例增强后呈中度或高度强化.9例病灶在T1WI图像上呈等或稍低信号,在T2WI上呈稍高信号,8个病灶呈中度或高度强化.9个病灶内有条索状、片状的长T1、短T2区域,增强后未见强化.光镜下瘤细胞长梭形,无明显异型性,核分裂少见,呈侵袭性生长,浸润周围的横纹肌及脂肪组织,伴胶原纤维增生及程度不等的透明变性、黏液变性.结论 韧带样型纤维瘤影像学变现具有一定的特异性,认识其病理学改变有助于解释该病的影像学表现,有助于提高术前诊断率,磁共振对手术切除范围的判断具有优势.

Imaging features and pathological findings of desmoid-type fibromatosis

Objective To study the imaging features and pathological basis of desmoid-type fibromatosis. Methods Imaging and pathological characteristics of 14 histologically confirmed desmoid-type fibromatosis were analyzed retrospectively. The imaging studies included CT and MR imaging without （3, 1） or with （3, 7） intravenous contrast enhancement. Results The mean size of the tumors was 6.2 cm. Of the 16 tumors, 9 had ill-defined margins, 11 had lobulated or irregular contours. On CT, 7/8 tumors were hypo- or iso-dense; 3 tumors showed moderate to intense contrast enhancement. All 9 tumors were iso- or slightly hyperintense on T1W MR imaging and hyperintense on T2W imaging. All 8 tumors showed moderate to intense gadolinium enhancement with linear and curvilinear non- enhancing areas of low signal intensities on T1WI or T2WI. The tumor was composed of spindle cells surrounded by variable amount of collagen bundles on light microscopy. There was no nuclear atypia or mitosis. The collagen fibers showed hyaline and mucinous degeneration. Conclusion The imaging features of desmoid-type fibromatosis correlate well with pathological appearance. MRI can demonstrate local tumor spread better than CT for treatment planning.