Department of Pathology, Tachikawa General Hospital, Nagaoka, Japan.
Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan.
Virchows Arch. 2023 Jun;482(6):1085-1089. doi: 10.1007/s00428-023-03505-w. Epub 2023 Feb 9.
Hepatic small vessel neoplasia (HSVN) is a recently recognized hemangioma of the liver with uncertain malignant potential. Almost all the patients are asymptomatic. Budd-Chiari syndrome (BCS) is a rare disorder characterized by noncardiogenic hepatic venous outflow obstruction. Benign hepatocellular nodules have been acknowledged for a long time in the liver with the chronic BCS. However, there has been no case report of BCS associated with HSVN. The patient was diagnosed with BCS 13 years ago. The imaging test initially displayed multiple hepatic nodules that were suspected of benign hepatocellular nodules. They gradually increased in size and number in the course of the disease. At an autopsy, these nodules were confirmed to be multifocal HSVN. The tumor of the present case could not be proved to have GNAQ and GNQ14 mutations. We describe the case focusing on the chronological imaging changes and discuss on the relationship between BCS and HSVN.
肝小血管肿瘤(HSVN)是一种新近被认识的肝脏血管瘤,其恶性潜能不确定。几乎所有患者均无症状。布加综合征(BCS)是一种罕见的疾病,其特征为非心源性肝静脉流出道阻塞。慢性布加综合征患者的肝脏中很早就被承认存在良性肝细胞结节。然而,目前还没有 BCS 与 HSVN 相关的病例报告。该患者在 13 年前被诊断为 BCS。影像学检查最初显示多个肝结节,疑似良性肝细胞结节。在疾病过程中,这些结节的大小和数量逐渐增加。在尸检中,这些结节被证实为多灶性 HSVN。目前的病例肿瘤不能被证明存在 GNAQ 和 GNQ14 突变。我们描述了这一病例,重点关注了其随时间变化的影像学改变,并讨论了 BCS 和 HSVN 之间的关系。