History: Adult presents with abdominal pain.
病史：成人患者，表现为腹部疼痛。
CT images are shown below. CT图像如下所示。
Additional clinical history: The patient is status post multiple small-bowel resections and subtotal colectomy.
病史补充：患者曾行部分小肠及结肠切除术。
Below are images from the pelvis.
盆腔CT图像如下所示。
Findings
There are multiple soft-tissue masses in the anterior abdominal muscles. There is some enhancement of the most dominant mass extending down the inferior rectus abdominus muscle.
影像表现：前腹壁肌肉内见多发软组织肿物，最明显的肿物延伸至腹直肌下部，增强扫描有强化。
Differential diagnosis — mesenteric masses
- Desmoid tumor
- Carcinoid tumor
- Lymphoma
- Omental or mesenteric metastases
- Fibrosing mesenteritis
- Hematoma
- Rhabdomyosarcoma
- Liposarcoma
- Gastrointestinal stromal tumors
鉴别诊断（肠系膜肿物）：
- 韧带样纤维瘤（硬纤维瘤）
- 类癌
- 淋巴瘤
- 网膜或肠系膜转移
- 纤维性肠系膜炎
- 血肿
- 横纹肌肉瘤
- 脂肪肉瘤
- 胃肠道间质瘤
Diagnosis: Desmoid tumors
诊断：韧带样纤维瘤
Key points
Desmoid tumors
韧带样纤维瘤（硬纤维瘤）
Desmoid tumors are benign proliferations of fibroblastic cells in connective or fibrous tissue. 韧带样纤维瘤是结缔组织或纤维组织的纤维母细胞的良性增殖。
They are most commonly found in the abdomen, arising from the anterior abdominal wall, mesentery, or retroperitoneum. 最多见于腹部，起源于前腹壁、肠系膜或腹膜后。
They are locally aggressive and have a high recurrence rate. 具有局部侵袭性，复发率高。
75% of patients have had prior abdominal surgery. 75%的患者之前有腹部手术史。
18% to 20% of patients with Gardner\’s syndrome have it. Patients have a mutation in the APC gene. Gardner综合征（遗传性肠息肉综合征）中18%~20%的患者可患此病，其APC基因有变异。
Can be resistant to percutaneous biopsy because very hard. 由于肿瘤非常硬，不易穿刺活检。
Sporadically occurring tumors are more numerous than those seen in familial adenomatous polyposis (FAP) . 较家族性腺瘤性息肉病的发病患者而言，散发肿瘤更加多见。
The exact cause is unknown. 具体病因不明。
Best diagnostic clue is a small-bowel mesentery or abdominal wall mass arising from a scar of prior surgery. 最好的诊断线索为起源于先前手术瘢痕的小肠系膜或腹壁肿物。
Location: 发病部位
Small-bowel mesentery, abdominal wall musculature, retroperitomeum, pelvic musculature 小肠系膜、腹壁肌肉组织、腹膜后、盆部肌肉组织
Extraabdominal locations: bladder, ribs, pelvic bones 腹部以外：膀胱、肋骨、骨盆
Range in size from 5 cm to 20 cm. 病变大小范围为5~20cm。
Can be solitary or multiple. 可单发或多发。
Radiologic overview of the diagnosis ：影像诊断综述
Ultrasonography: Appear with variable echogenicity, with smooth, well-defined margins. 超声：表现为回声不均，边界光滑、清楚；
CT: May enhance and have either ill- or well- defined margins. Encase or displace mesenteric vessels. CT：有强化，边界欠清或清晰，可包裹或推移肠系膜血管；
MRI: Low signal intensity relative to muscle on T1-weighted images and variable signal intensity on T2-weighted images. There are no specific imaging features to distinguish desmoid tumors from other solid masses. MRI：T1WI上呈低于肌肉的较低信号，T2WI上呈不均质高信号。没有特征性影像表现可与其它实性肿物相鉴别。
Management and treatment 处理及治疗
Wide surgical resection 广泛性手术切除
Radiation therapy for abdominal wall 腹壁放疗
Steroids, nonsteroidal anti-inflammatory drugs (NSAIDs), chemotherapy, among other medications 类固醇，非类固醇抗炎药，化疗，其他药物治疗
May require small-bowel transplant 可能需要小肠移植
Recurrence rates of 25% to 65% 复发率为25%~65%
Key points 关键点
- No specific imaging features to distinguish desmoid tumors from other solid masses. 没有特征性影像表现可将韧带样纤维瘤与其他实性肿物相鉴别。
- Most often seen in abdominal wall and small-bowel mesentery. 最常见于腹壁及小肠系膜。
- Higher prevalence in patients with Gardner\’s syndrome. 在Gardner综合征（遗传性肠息肉综合征）患者中有较高的发病率。
- Has a high recurrence rate. 复发率高。
- The tumors are benign and rarely metastasize. 良性肿瘤，极少发生转移。
- Can cause complications from mass effect. 可因占位效应导致相应并发症。
- Most effective treatment of accessible and smaller desmoid tumors is surgical resection with negative margins, although it may not prevent local recurrence. 易触摸的及较小的韧带样纤维瘤最有效的治疗方法为手术切除，尽管在切缘阴性的情况下也不能阻止肿瘤局部复发。
