Zhang Zhong-lin, Liang Chang-hong, Liu Yu-bao, Xie Shu-fei, Yu Yuan-xin, Wang Qiu-shi, Liu Zai-yi, Li Jing-lei
Department of Radiology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou 510080, China.
Nan Fang Yi Ke Da Xue Xue Bao. 2010 Nov;30(11):2495-7.
To explore the computed tomography (CT) and magnetic resonance imaging (MRI) features of desmoid-type fibromatosis, and improve the diagnostic accuracy and understanding of the disease.
The CT and MRI features of 18 cases of surgically and pathologically confirmed desmoid-type fibromatosis were reviewed retrospectively. Among the patients, 10 received CT pre- and post-contrast scanning, and 8 patients had MRI pre- and post-contrast scanning. The CT and MRI features were analyzed in comparison with the pathological findings.
In the extra abdominal cases, the tumors occurred in the head and neck in 3, in the dorsal part of the chest in 2, in the abdominal wall and groin area in 9, and in the peritoneal cavity in 4; concomitant Gardner syndrome was found in 1 case. In 4 cases the tumor occurred within 1 to 3 years after abdominal surgeries. Pathologically, the lesion was hard and composed of fusiform fibroblasts and myofibroblast. The cells showed no obvious heteromorphism with few karyokinesis, growing invasively and recurrent locally but without distant metastasis. Immunohistochemically, the fibroblasts and myofibroblasts expressed vimentin, and the myofibroblasts were positive for SMA. On CT and MRI, the lesion appeared benign with malignant growth pattern, and caused compression of the adjacent organs and vessels or encasement of the vessels; the border was unclear without encapsulation, and necrosis and calcification was scarce. The density and signal of the tumor were well distributed. Twelve patients displayed obvious enhancement and 5 showed uneven enhancement.
The CT and MRI features of desmoid-type fibromatosis are characteristic, and CT and MRI are valuable modalities for the diagnosis and differential diagnosis of the tumor.
探讨韧带样型纤维瘤病的计算机断层扫描（CT）及磁共振成像（MRI）特征，提高对该病的诊断准确性及认识。
回顾性分析18例经手术及病理证实的韧带样型纤维瘤病患者的CT及MRI表现。其中10例行CT平扫及增强扫描，8例行MRI平扫及增强扫描。将CT及MRI表现与病理结果进行对比分析。
腹外病例中，肿瘤发生于头颈部3例，胸背部2例，腹壁及腹股沟区9例，腹腔内4例；1例合并Gardner综合征。4例肿瘤发生于腹部手术后1至3年。病理上，病变质地硬，由梭形成纤维细胞及肌成纤维细胞组成。细胞异型性不明显，核分裂象少见，呈浸润性生长，局部复发但无远处转移。免疫组化显示，成纤维细胞及肌成纤维细胞表达波形蛋白，肌成纤维细胞平滑肌肌动蛋白（SMA）阳性。CT及MRI表现为良性病变但呈恶性生长方式，可压迫相邻器官及血管或包绕血管；边界不清，无包膜，坏死及钙化少见。肿瘤密度及信号均匀。12例表现为明显强化，5例强化不均匀。
韧带样型纤维瘤病的CT及MRI表现具有特征性，CT及MRI对该肿瘤的诊断及鉴别诊断具有重要价值。
