Liver adenomatosis:: Reappraisal, diagnosis, and surgical management -: Eight new cases and review of the literature
Liver adenomatosis:: Reappraisal, diagnosis, and surgical management -: Eight new cases and review of the literature
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DOI：
10.1097/00000658-200001000-00011
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发表时间：
2000-01-01
影响因子：
9
通讯作者：
Gignoux, M
中科院分区：
文献类型：
--
作者：
Chiche, L;Dao, T;Gignoux, M
目的肝腺瘤病（liver adenomatosis， LA）是一种罕见的疾病，最初由Flejou等人于1985年从一系列13例病例中定义。一九九八年，共有38宗个案可供分析，其中包括8宗个人个案。本研究的目的是回顾和重新评估这种罕见肝脏疾病的特征，并讨论诊断和治疗方案。背景：la被定义为在正常实质内出现bbbb10腺瘤。既没有女性的优势，也没有注意到与雌激素/孕激素摄入量的关系。人们对自然进程知之甚少。方法对8例新发病例的临床表现、发展、组织学特征、治疗方案和结果进行分析。结果从诊断的角度来看，肝腺瘤病可分为两种不同的表现和发展形式：大块型和多灶型。雌激素和黄体酮的作用被重新评估。出血和恶性转化的风险是主要关注的。在作者的系列研究中，有两名年轻女性的肝移植是巨大的，侵袭性的，并获得了良好的结果。结论肝腺瘤病是一种罕见的疾病，多见于女性，其结局和进化各不相同，并因雌激素的摄入而加重。大多数情况下，需要保守手术。肝移植仅适用于高度症状和侵袭性疾病。
ObjectiveLiver adenomatosis (LA) is a rare disease originally defined by Flejou et al in 1985 from a series of 13 cases. In 1998, 38 cases were available for analysis, including eight personal cases. The aim of this study was to review and reappraise the characteristics of this rare liver disease and to discuss diagnosis and therapeutic options.BackgroundLA was defined as the presence of >10 adenomas in an otherwise normal parenchyma. Neither female predominance nor a relation with estrogen/progesterone intake has been noted. Natural progression is poorly known.MethodsThe clinical presentation, evolution, histologic characteristics, and therapeutic options and results were analyzed based on a personal series of eight new cases and an updated review of the literature.ResultsFrom a diagnostic standpoint, two forms of liver adenomatosis with different presentations and evolution can be defined: a massive form and a multifocal form. The role of estrogen and progesterone is reevaluated. The risks of hemorrhage and malignant transformation are of major concern. In the authors' series, liver transplantation was indicated in two young women with the massive, aggressive form, and good results were obtained.ConclusionLiver adenomatosis is a rare disease, more common in women, where outcome and evolution vary and are exacerbated by estrogen intake. Most often, conservative surgery is indicated. Liver transplantation is indicated only in highly symptomatic and aggressive forms of the disease.