Yan Lifen, Huang Runqian, Chen Shuting, Chen Jiawei, Li Jinglei
Department of Radiology, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, China.
Department of Radiology, People's Hospital of Yingde City, Yingde, Guangdong, China.
Front Oncol. 2024 Nov 21;14:1490717. doi: 10.3389/fonc.2024.1490717. eCollection 2024.
Primary hepatic leiomyosarcoma (PHLS) is an extremely rare malignant tumor, which is often elusive in early diagnosis due to its rarity and nonspecific clinical and imaging presentations. Herein, we present a case of PHLS in a 66-year-old male and a review of the English literature from January 2000 to December 2023, focusing on the clinical and imaging characteristics of 30 patients with PHLS. The present patient was admitted to our hospital with complaints of abdominal distension, with history of hepatitis B. Tumor markers, including alpha-fetoprotein, carcinoembryonic antigen, and CA 19-9, were within the normal range. A hepatic tumor was incidentally identified during an abdominal ultrasound examination, further evaluated by contrast-enhanced CT and MR scans, which was preliminarily misdiagnosed as hepatocellular carcinoma. The tumor was surgically excised and definitively diagnosed as PHLS, characterized by two distinct areas with varying imaging features on contrast-enhanced CT and MR images. PHLS typically manifests as a well-defined, heterogeneously hypo- or iso-dense mass on CT, with a slightly prolonged T2 signal on MRI, and exhibits gradual enhancement during dynamic contrast-enhanced imaging. We advocate that the possibility of PHLS should be considered when the aforementioned imaging features are observed.
原发性肝平滑肌肉瘤(PHLS)是一种极为罕见的恶性肿瘤,由于其罕见性以及非特异性的临床和影像学表现,早期诊断往往具有挑战性。在此,我们报告一例66岁男性的PHLS病例,并对2000年1月至2023年12月的英文文献进行综述,重点关注30例PHLS患者的临床和影像学特征。该患者因腹胀入院,有乙肝病史。包括甲胎蛋白、癌胚抗原和CA 19-9在内的肿瘤标志物均在正常范围内。腹部超声检查偶然发现肝脏肿瘤,经增强CT和磁共振成像扫描进一步评估,最初被误诊为肝细胞癌。肿瘤经手术切除,最终确诊为PHLS,其特征是在增强CT和磁共振图像上有两个具有不同影像学特征的区域。PHLS在CT上通常表现为边界清晰、密度不均匀减低或等密度的肿块,在MRI上T2信号略有延长,在动态增强成像过程中呈渐进性强化。我们主张,当观察到上述影像学特征时,应考虑PHLS的可能性。