【病例】腹壁硬纤维瘤1例CT及MR影像表现

10 1月

History: A 35-year-old woman presents with an abdominal lump. The patient has a history of cesarean section and a family history of breast cancer and colon cancer.

病史:35岁女性,发现腹部肿块;有剖腹产手术史,有乳腺癌及结肠癌家族史。

A CT scan of the abdomen was performed.

行腹部CT扫描

【病例】腹壁硬纤维瘤1例CT及MR影像表现

【病例】腹壁硬纤维瘤1例CT及MR影像表现

【病例】腹壁硬纤维瘤1例CT及MR影像表现

The patient also underwent ultrasound and MRI examinations.

患者也行超声及MRI检查。

【病例】腹壁硬纤维瘤1例CT及MR影像表现

【病例】腹壁硬纤维瘤1例CT及MR影像表现

T1-weighted volumetric interpolated breath-hold examination (VIBE) fat-suppressed (FS) pre- and postcontrast MR images:

T1WI容积内插屏气检查(VIBE)脂肪抑制(FS)序列平扫及增强MR图像

【病例】腹壁硬纤维瘤1例CT及MR影像表现

【病例】腹壁硬纤维瘤1例CT及MR影像表现

T2-weighted half-Fourier acquisition single-shot turbo spin-echo (HASTE) and HASTE FS MR images:

T2WI半傅里叶采集单发射快速自旋回波(HASTE)及HASTE压脂MR图像

【病例】腹壁硬纤维瘤1例CT及MR影像表现

【病例】腹壁硬纤维瘤1例CT及MR影像表现

T1-weighted VIBE FS pre- and postcontrast MR images:

T1WI VIBE压脂平扫及增强MR图像

【病例】腹壁硬纤维瘤1例CT及MR影像表现

【病例】腹壁硬纤维瘤1例CT及MR影像表现

Findings

CT: CT demonstrates a right rectus abdominis muscle soft-tissue density ovoid lesion measuring approximately 4.7 x 3.5 x 2.3 cm.

Ultrasound: Subcutaneous tissues demonstrate an ovoid hypoechoic mass with peripheral areas of increased echogenicity measuring 3.6 x 3.5 x. 2.4 cm with some peripheral vascularity.

MRI: MRI demonstrates a 4.9 x.3.7 x 2.6-cm ovoid lesion within the right rectus abdominis with additional more focal 2.2-cm linear inferior extension. The lesion shows low signal on T1-weighted imaging, heterogenous low signal on T2-weighted imaging demonstrating heterogenous enhancement. There is no extension of the mass into the fat tissues superficial to the abdominis rectus fascia or the intraabdominal tissues deep to the rectus fascia.

影像表现:

CT:右侧腹直肌见一大小约4.7 x 3.5 x 2.3 cm的卵圆形软组织密度肿物;

超声:皮下组织内见一卵圆形低回声肿物,外周区域回声增强并见外周血管;

MRI:右侧腹直肌卵圆形肿物,病灶局部呈线形向下延伸约2.2cm。T1WI上呈低信号,T2WI上呈不均质低信号,增强扫描不均质强化。肿物未侵犯腹直肌浅侧的脂肪组织及深面的腹腔内组织。

Differential diagnosis

  • Desmoid tumor
  • Soft-tissue sarcoma
  • Lymphoma
  • Trauma/hematoma

鉴别诊断:

  • 硬纤维瘤
  • 软组织肉瘤
  • 淋巴瘤
  • 外伤/血肿

Diagnosis: Right-sided abdominal wall desmoid tumor, biopsy-proven

最后诊断:右侧腹壁硬纤维瘤(穿刺证实)

Discussion

Desmoid tumors

硬纤维瘤

Desmoid tumors are rare tumors that arise from fibroblasts. They are benign but may be locally aggressive. 硬纤维瘤是起源于纤维母细胞的罕见肿瘤,良性,但可有局部侵袭性。

They are most common in women of childbearing age. 多见于育龄期女性。

Patients may be asymptomatic or present with abdominal pain or an abdominal mass. 患者可无症状或表现为腹部疼痛或腹部肿物。

Histologically, they resemble low-grade fibrosarcomas.组织学上,类似低级别纤维肉瘤。

They can be located intra- or extra-abdominally. 肿瘤可位于腹壁内或腹壁外。

  • Intra-abdominal tumors account for two-thirds of cases. 2/3的肿瘤位于腹壁内;
  • One-third of extra-abdominal desmoids tumors are located in the shoulders and upper extremities. 1/3的腹壁外硬纤维瘤位于肩部及上肢。

Mesenteric desmoids tumors may compress adjacent small bowel, leading to small-bowel obstruction. 肠系膜硬纤维瘤可压迫邻近小肠,导致小肠梗阻。

Rectus or oblique muscles are most commonly involved in abdominal wall desmoids. 腹壁硬纤维瘤最常累及腹直肌或腹斜肌。

Most are sporadic but may be associated with Gardner syndrome or familial adenomatous polyposis (FAP). 大部分为散发病例,一部分可与Gardner综合征或家族性腺瘤性息肉病(FAP)相关。

Other associations include oral contraceptives, trauma, and prior abdominal surgery (75% of cases). 其他相关因素包括:口服避孕药,外伤,腹壁手术史(75%的病例与此有关)。

Imaging findings: 

CT findings: A desmoid tumor appears well-defined, hyperdense to muscle on nonenhanced CT, and hypoenhancing on contrast-enhanced CT.

Ultrasound findings: It appears as a well-defined lesion with variable echogenicity.

MRI findings: The tumor will have heterogeneously high signal on T2-weighted/short tau inversion-recovery (STIR) sequences, appear homogenously hypo-/isointense on T1-weighted imaging, and will have bands of internal low signal on all sequences. Higher T2 signal generally correlates with more locally aggresive tumor.

影像表现:

  • CT表现:边界清楚,平扫较肌肉密度略高,增强扫描弱强化;
  • 超声:边界清楚,回声高低表现不一;
  • MRI表现:T2WI压脂(STIR)上呈不均质高信号,T1WI上呈均匀低或等信号,所有序列上均可见内部带状低信号;更高的T2信号通常与肿瘤的局部侵袭有一定相关性。

Treatment: Patients who are asymptomatically stable should be followed with serial imaging. For symptomatic unresectable cases, treatment is surgical resection or radiation/systemic therapy. 治疗:无症状的较为稳定的患者应当影像随访;对于有症状的手术无法切除的病例,治疗应采取手术切除或放疗/系统治疗。

Recurrence post surgery is very common. 术后复发很常见。


资料补充:

Gardner综合征

是一种罕见的家族性腺瘤性息肉病亚型,为常染色体显性遗传病;

典型的 Gardner综合征以三联症出现,表现为结肠多发息肉( 数以万计) 并有息肉潜在恶变、多发扁骨骨疣、多样的皮肤组织肿物;

硬纤维瘤是Gardner综合征最主要的肠外病变,也是该病第二位死因;硬纤维瘤属于良性肿瘤,但有局部浸润倾向,术后易复发,少数亦可先于肠道息肉出现。由于硬纤维瘤的侵袭生长及好发于肠系膜根部,目前对无肠梗阻或严重肾盂积水等并发症的腹腔内硬纤维瘤,不主张手术。早期诊断对于患者的治疗方式及预后至关重要。

【病例】腹壁硬纤维瘤1例CT及MR影像表现

结肠见多发息肉;腹腔硬纤维瘤:左侧腹腔软组织肿块( 黑箭) ,与胃壁、脾门及胰尾分界不清,增强扫描动脉期、静脉期肿块均呈轻度不均匀强化。

来源:Gardner综合征一例. 杨维森(临床放射学杂志2015年)


关于名称:

硬纤维瘤由 Muller于1938年命名,因其具有侵袭性,又称侵袭性纤维瘤、韧带样纤维瘤或腹和腹外纤维样肿瘤等。

发表回复

您的电子邮箱地址不会被公开。