Shi Zhuo, Zhao Xin-Ming, Jiang Jiu-Ming, Li Meng, Xie Li-Zhi
Department of Imaging Diagnosis, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China.
GE Healthcare China, Beijing 100176, China.
World J Clin Cases. 2021 Oct 16;9(29):8710-8717. doi: 10.12998/wjcc.v9.i29.8710.
Desmoid fibroma is a rare soft tissue tumor originating from the aponeurosis, fascia, and muscle, and it is also known as aponeurotic fibroma, invasive fibroma, or ligamentous fibroma.
To investigate the clinical and imaging features of desmoid tumors of the extremities.
Thirteen patients with desmoid fibroma of the extremities admitted to our hospital from October 2016 to March 2021 were included. All patients underwent computed tomography (CT), magnetic resonance imaging (MRI), and pathological examination of the lesion. Data on the diameter and distribution of the lesion, the relationship between the lesion morphology and surrounding structures, MRI and CT findings, and pathological features were statistically analyzed.
The lesion diameter ranged from 1.7 to 8.9 cm, with an average of 5.35 ± 2.39 cm. All lesions were located in the deep muscular space, with the left and right forearm each accounting for 23.08% of cases. Among the 13 patients with desmoid fibroma of the extremities, the lesions were "patchy" in 1 case, irregular in 10, and quasi-round in 2. The boundary between the lesion and surrounding soft tissue was blurred in 10 cases, and the focus infiltrated along the tissue space and invaded the adjacent structures. Furthermore, the edge of the lesion showed "beard-like" infiltration in 2 cases; bone resorption and damage were found in 8, and bending of the bone was present in 2; the boundary of the focus was clear in 1. According to the MRI examination, the lesions were larger than 5 cm (61.54%), round or fusiform in shape (84.62%), had an unclear boundary (76.92%), showed uniform signal (69.23%), inhomogeneous enhancement (84.62%), and "root" or "claw" infiltration (69.23%). Neurovascular tract invasion was present in 30.77% of cases. CT examination showed that the desmoid tumors had slightly a lower density (69.23%), higher enhancement (61.54%), and unclear boundary (84.62%); a CT value < 50 Hu was present in 53.85% of lesions, and the enhancement was uneven in 53.85% of cases. Microscopically, fibroblasts and myofibroblasts were arranged in strands and bundles, without obvious atypia but with occasional karyotyping; cells were surrounded by collagen tissue. There were disparities in the proportion of collagen tissue in different regions, with abundant collagen tissue and few tumor cells in some areas, similar to the structure of aponeuroses or ligaments, and tumor cells invading the surrounding tissues.
Desmoid tumors of the extremities have certain imaging features on CT and MRI. The two imaging techniques can be combined to improve the diagnostic accuracy, achieve a comprehensive diagnosis of the disease in the clinical practice, and reduce the risk of missed diagnosis or misdiagnosis. In addition, their use can ensure timely diagnosis and treatment.
硬纤维瘤是一种罕见的软组织肿瘤，起源于腱膜、筋膜和肌肉，也被称为腱膜纤维瘤、侵袭性纤维瘤或韧带样纤维瘤。
探讨四肢硬纤维瘤的临床及影像学特征。
纳入2016年10月至2021年3月我院收治的13例四肢硬纤维瘤患者。所有患者均接受了计算机断层扫描（CT）、磁共振成像（MRI）及病变的病理检查。对病变的直径及分布、病变形态与周围结构的关系、MRI和CT表现及病理特征等数据进行统计学分析。
病变直径为1.7～8.9 cm，平均为5.35±2.39 cm。所有病变均位于深部肌肉间隙，左右前臂各占23.08%。13例四肢硬纤维瘤患者中，病变呈“斑片状”1例，不规则形10例，类圆形2例。10例病变与周围软组织边界不清，病灶沿组织间隙浸润并侵犯相邻结构。此外，2例病变边缘呈“胡须样”浸润；8例出现骨质吸收和破坏，2例出现骨质弯曲；1例病灶边界清晰。根据MRI检查，病变大于5 cm者占61.54%，呈圆形或梭形者占84.62%，边界不清者占76.92%，信号均匀者占69.23%，不均匀强化者占84.62%，呈“根”或“爪”样浸润者占69.23%。30.77%的病例存在神经血管束侵犯。CT检查显示硬纤维瘤密度略低者占69.23%，强化较高者占61.54%，边界不清者占84.62%；53.85%的病变CT值<50 Hu，53.85%的病例强化不均匀。显微镜下，成纤维细胞和肌成纤维细胞呈束状排列，无明显异型性，但偶见核型；细胞被胶原组织包绕。不同区域胶原组织比例存在差异，部分区域胶原组织丰富而肿瘤细胞少，类似腱膜或韧带结构，肿瘤细胞侵犯周围组织。
四肢硬纤维瘤在CT和MRI上有一定的影像学特征。两种影像学技术联合应用可提高诊断准确性，在临床实践中实现对疾病的全面诊断，降低漏诊或误诊风险。此外，其应用可确保及时诊断和治疗。
