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下腔静脉平滑肌肉瘤:横断面成像的诊断特征。

Leiomyosarcomas of the inferior vena cava: diagnostic features on cross-sectional imaging.

机构信息

Department of Clinical Radiology, The Royal Marsden NHS Foundation Trust, London, UK.

出版信息

Clin Radiol. 2011 Jan;66(1):50-6. doi: 10.1016/j.crad.2010.08.004.

Abstract

AIM

To evaluate the cross-sectional radiological appearances and to review the clinical presentation and outcome of patients with leiomyosarcomas of the inferior vena cava (IVC LMS). These are rare aggressive tumours that present late with non-specific symptoms and have a poor prognosis.

MATERIALS AND METHODS

From January 2002 to December 2008, the radiological images of 23 sequential patients with pathologically proven IVC LMS were independently reviewed by two experienced radiologists. The clinical presentation, treatment including surgical details, and outcome were recorded.

RESULTS

There were 19 females and four males with a mean age of 53 years. CT typically demonstrated a large, lobulate, non-calcified heterogeneous mass with peripheral enhancement. T1-weighted magnetic resonance imaging (MRI) images demonstrated a mass with a low signal intensity and T2-weighted MRI images demonstrated a mass with a high signal intensity. Clinical presentation included leg oedema, back and abdominal pain with almost 50% of patients presenting with metastases. Eleven patients underwent ablative surgery. The mean survival time of all patients in the study was 34 months and that of the 11 post-surgical patients was 56 months.

CONCLUSION

There are a variety of diagnostic features on both computed tomography (CT) and MRI which aid the diagnosis of this unusual vascular neoplasm. CT is vital in determining the location of the tumour within the IVC and MRI accurately depicts its extent and the potential for surgical resectability, which offers the only chance of survival.

摘要

目的

评估下腔静脉平滑肌肉瘤(IVC LMS)的横断面影像学表现,并回顾其临床表现和转归。这些罕见的侵袭性肿瘤通常症状不典型且较晚出现,预后较差。

材料与方法

2002 年 1 月至 2008 年 12 月,23 例经病理证实的 IVC LMS 患者的影像学资料由两位经验丰富的放射科医生独立进行回顾性分析。记录患者的临床表现、治疗方法(包括手术细节)及转归。

结果

23 例患者中,女性 19 例,男性 4 例,平均年龄 53 岁。CT 表现为边界不清的大而分叶状、非钙化的混杂密度肿块,周边强化。T1 加权磁共振成像(MRI)显示为低信号强度肿块,T2 加权 MRI 显示为高信号强度肿块。临床表现包括下肢水肿、腰背及腹部疼痛,近 50%的患者有转移病灶。11 例行消融手术。本研究中所有患者的平均生存时间为 34 个月,11 例手术后患者的平均生存时间为 56 个月。

结论

CT 和 MRI 均有多种有助于诊断这种罕见血管肿瘤的特征性表现。CT 对确定肿瘤在 IVC 中的位置至关重要,MRI 则能准确描绘肿瘤的范围和手术可切除性,这是唯一的生存机会。

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