Boccatonda Andrea, Marcellini Marco Marcello, Ruggeri Eugenio, Felicani Cristina, Brighenti Alice, Loiacono Rossella, Ercolani Giorgio, Serra Carla
Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola-Malpighi, via Massarenti n 9, 40138, Bologna, Italy.
Medicina interna e diabetologia, Ospedale Infermi Rimini, AUSL Romagna, Rimini, Italy.
J Ultrasound. 2025 Mar;28(1):261-268. doi: 10.1007/s40477-024-00973-x. Epub 2024 Nov 18.
Perivascular epithelioid cell neoplasms (PEComas) and epithelioid angiomyolipomas (EAMLs) are two different denominations for the same "mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells". Hepatic PEComa/EAML is a very rare neoplasm, and only 29 case reports of hepatic PEComa and 25 of hepatic EAML have been reported in the current literature. A clear female predominance with a mean age at diagnosis of 42.5 years old can be observed by literature review. Ultrasound (US) examination was the first-line diagnostic technique in most of the cases of hepatic PEComa, but it was documented in very few cases of hepatic EAML. A great variability in the ultrasonographic B-mode, color Doppler and contrast-enhanced ultrasonography (CEUS) features of hepatic PEComa/EAML emerges. Computed tomography and magnetic resonance were the most common used techniques to confirm the nature of the hepatic lesion, even if the anatomo-pathological examination was the only technique to display a certainty diagnosis and to differentiate hepatic PEComa/EAML from benign and malignant hepatic lesions. The great majority of hepatic PEComas/EAMLs are surgically treated without any adjuvant therapy.
血管周上皮样细胞瘤(PEComas)和上皮样血管平滑肌脂肪瘤(EAMLs)是同一“由组织学和免疫组织化学上独特的血管周上皮样细胞组成的间充质肿瘤”的两种不同命名。肝脏PEComa/EAML是一种非常罕见的肿瘤,目前文献中仅报道了29例肝脏PEComa病例和25例肝脏EAML病例。通过文献回顾可以发现,女性明显居多,诊断时的平均年龄为42.5岁。在大多数肝脏PEComa病例中,超声(US)检查是一线诊断技术,但在极少数肝脏EAML病例中有记录。肝脏PEComa/EAML的超声B超、彩色多普勒和超声造影(CEUS)特征存在很大差异。计算机断层扫描和磁共振是最常用于确认肝脏病变性质的技术,尽管解剖病理学检查是唯一能明确诊断并将肝脏PEComa/EAML与肝脏良恶性病变区分开来的技术。绝大多数肝脏PEComas/EAMLs通过手术治疗,无需任何辅助治疗。