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肝脏血管平滑肌脂肪瘤的影像学表现:关键特征与误诊防范。

Imaging hepatic angiomyolipomas: key features and avoiding errors.

机构信息

Radiology Department, Royal Perth Hospital, Perth, Australia.

Radiology Department, Austin Health, Heidelberg, Melbourne, Australia.

出版信息

Clin Radiol. 2020 Feb;75(2):88-99. doi: 10.1016/j.crad.2019.09.135. Epub 2019 Oct 31.

Abstract

Hepatic angiomyolipomas (HAMLs) are rare mesenchymal neoplasms, which have highly variable imaging appearances, often leading to misdiagnosis. They belong to the family of perivascular epithelioid cell neoplasms (PEComas). HAMLs have a wide spectrum of imaging appearances due to variable amounts of smooth muscle cells, adipose tissue, and blood vessels in their makeup. Although typically sporadic, they are also associated with tuberous sclerosis. Sporadic lesions tend to be solitary whilst patients with tuberous sclerosis often have multiple HAMLs invariably accompanied by renal AMLs. Having been originally considered benign hamartomas, increasing reports of complications, including malignant behaviour, has also resulted in uncertainty in regard to their optimal management. Typically described imaging characteristics are of a hypervascular fat-containing lesion with prominent intratumoural vessels and an early draining vein; however HAMLs commonly demonstrate a paucity of fat or wash-out on contrast-enhanced imaging, and not all HAML lesions are hypervascular. HAMLs can therefore easily be misdiagnosed as other hepatic lesions, in particular hepatocellular carcinoma. This review describes the imaging characteristics of HAMLs, illustrating the wide variety of potential appearances across ultrasound, contrast-enhanced ultrasound, computed tomography, and magnetic resonance imaging, and highlights the challenges and potential errors that can be made. This review will aid radiologists in avoiding potentially major pitfalls when faced with this rare but important liver pathology.

摘要

肝血管平滑肌脂肪瘤(HAML)是一种罕见的间叶性肿瘤,其影像学表现具有高度异质性,常导致误诊。它们属于血管周上皮样细胞肿瘤(PEComa)家族。由于平滑肌细胞、脂肪组织和血管在组成上的不同,HAML 的影像学表现具有广泛的谱。尽管通常为散发性,但也与结节性硬化症有关。散发性病变通常为单发,而结节性硬化症患者常有多个 HAML,通常伴有肾血管平滑肌脂肪瘤。HAML 最初被认为是良性错构瘤,但越来越多的并发症报告,包括恶性行为,也导致了对其最佳管理的不确定性。典型的影像学特征是富血管的含脂肪病变,伴有明显的肿瘤内血管和早期引流静脉;然而,HAML 在对比增强成像上通常表现为脂肪缺乏或洗脱,并非所有 HAML 病变均为富血管性。因此,HAML 很容易被误诊为其他肝脏病变,特别是肝细胞癌。本综述描述了 HAML 的影像学特征,说明了在超声、超声造影、计算机断层扫描和磁共振成像上的各种潜在表现,并强调了在面对这种罕见但重要的肝脏病变时可能出现的挑战和潜在错误。本综述将有助于放射科医生在面对这种罕见但重要的肝脏病变时避免潜在的重大陷阱。

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