Wanless I R, Albrecht S, Bilbao J, Frei J V, Heathcote E J, Roberts E A, Chiasson D
Department of Pathology, Toronto Western Hospital, Ontario.
Mod Pathol. 1989 Sep;2(5):456-62.
Focal nodular hyperplasia (FNH) is a lesion of the liver in which a large anomalous artery is located within a region of hyperplastic hepatic parenchyma. Patients with FNH commonly have other lesions, often vascular in nature, in the liver or other organs. We have noted that these associated lesions almost always occur in patients with multiple FNH. We therefore studied 27 autopsied patients with FNH. All 13 with multiple FNH had other lesions such as hemangioma of liver, meningioma, astrocytoma, telangiectasis of the brain, berry aneurysm, dysplastic systemic arteries, and portal vein atresia. One patient had several of these lesions including multiple FNH, meningioma, astrocytoma, vascular malformation of the brain stem, and hemangioma of the liver. In contrast, among the 14 patients with solitary FNH there were no associated lesions, except for hepatic hemangioma in one patient. The prevalence of this syndrome was estimated by examination of 2500 serial autopsies and autopsies with various components of the syndrome. On review of 73 consecutive autopsies with meningioma, three had multiple FNH, compared with seven of 2500 consecutive adult autopsies (P less than 0.001). Multiple FNH was found in two of 83 autopsies with astrocytoma (P less than 0.05) and in one of 139 autopsies with berry aneurysm (not significant). We describe a telangiectatic subtype of FNH which occurs in this syndrome as well as in a minority of patients with solitary FNH. The existence and character of this syndrome suggest that there may be an underlying systemic abnormality in some patients having components of the syndrome. Investigation of patients with multiple FNH lesions may reveal significant treatable lesions.
局灶性结节性增生（FNH）是肝脏的一种病变，其中一条粗大的异常动脉位于增生的肝实质区域内。FNH患者通常在肝脏或其他器官有其他病变，这些病变往往具有血管性质。我们注意到，这些相关病变几乎总是发生在患有多个FNH的患者中。因此，我们研究了27例经尸检的FNH患者。所有13例患有多个FNH的患者都有其他病变，如肝血管瘤、脑膜瘤、星形细胞瘤、脑毛细血管扩张、浆果样动脉瘤、发育异常的全身动脉和门静脉闭锁。一名患者有几种这些病变，包括多个FNH、脑膜瘤、星形细胞瘤、脑干血管畸形和肝血管瘤。相比之下，在14例患有孤立性FNH的患者中，除了一名患者有肝血管瘤外，没有相关病变。通过检查2500例连续尸检以及患有该综合征各个组成部分的尸检来估计该综合征的患病率。在回顾73例连续的脑膜瘤尸检时，有3例患有多个FNH，而在2500例连续的成人尸检中有7例（P小于0.001）。在83例星形细胞瘤尸检中有2例发现多个FNH（P小于0.05），在139例浆果样动脉瘤尸检中有1例发现（无统计学意义）。我们描述了一种FNH的毛细血管扩张亚型，它既出现在该综合征中，也出现在少数孤立性FNH患者中。该综合征的存在和特征表明，一些患有该综合征组成部分的患者可能存在潜在的全身异常。对患有多个FNH病变的患者进行调查可能会发现可显著治疗的病变。
