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认识下腔静脉平滑肌肉瘤:CT影像学特征及三维重建的应用价值

Recognizing an IVC leiomyosarcoma: CT imaging hallmarks and the utility of 3D rendering.

作者信息

Rahmatullah Zahra F, Yasrab Mohammad, Kawamoto Satomi, Fishman Elliot K

机构信息

Johns Hopkins Hospital, Baltimore, United States.

出版信息

Abdom Radiol (NY). 2025 Aug 25. doi: 10.1007/s00261-025-05175-5.

Abstract

Inferior vena cava (IVC) leiomyosarcoma is a rare and aggressive malignancy arising from smooth muscle cells of the IVC wall, accounting for less than 1 in 100,000 adult malignancies. It carries a poor prognosis, with high recurrence rates and 5-year disease-free survival reported as low as 6%. Clinical presentation is often vague, including abdominal or back pain, lower extremity edema, and weight loss, making imaging pivotal for diagnosis. Contrast-enhanced CT (CECT) is the modality of choice, but diagnosis remains challenging due to overlap with more common pathologies such as renal cell carcinoma, hepatocellular carcinoma, adrenal tumors, and retroperitoneal lymphadenopathy. This pictorial review presents a curated series of cases highlighting the spectrum of imaging appearances of IVC leiomyosarcoma. By showcasing both typical and atypical imaging patterns, this review aims to broaden radiologists' diagnostic awareness and improve confidence in identifying this elusive tumor. Special emphasis is placed on 3D CT imaging techniques, including optimal acquisition protocols and post-processing methods, to enhance lesion visualization and vascular mapping. Early and accurate recognition is crucial, as aggressive surgical resection remains the mainstay of treatment. Understanding key imaging clues can facilitate timely diagnosis and intervention, ultimately impacting patient outcomes in this rare but life-threatening disease.

摘要

下腔静脉平滑肌肉瘤是一种罕见的侵袭性恶性肿瘤,起源于下腔静脉壁的平滑肌细胞,在成人恶性肿瘤中占比不到十万分之一。其预后较差,复发率高,据报道5年无病生存率低至6%。临床表现往往不明确,包括腹痛或背痛、下肢水肿和体重减轻,因此影像学检查对诊断至关重要。增强CT(CECT)是首选的检查方式,但由于与肾细胞癌、肝细胞癌、肾上腺肿瘤和腹膜后淋巴结病等更常见的病理情况存在重叠,诊断仍然具有挑战性。本影像学综述展示了一系列经过筛选的病例,突出了下腔静脉平滑肌肉瘤的影像学表现谱。通过展示典型和非典型的影像学模式,本综述旨在拓宽放射科医生的诊断意识,提高识别这种难以捉摸的肿瘤的信心。特别强调了三维CT成像技术,包括最佳采集协议和后处理方法,以增强病变可视化和血管成像。早期准确识别至关重要,因为积极的手术切除仍然是主要的治疗方法。了解关键影像学线索有助于及时诊断和干预,最终影响这种罕见但危及生命的疾病的患者预后。

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