Abstract:
Hepatic adenomatosis is a rare disease consisting of multiple adenomas in otherwise-normal liver parenchyma. Though the discovery of this entity goes back several years, its diagnosis is still challenging in terms of its definition and pathophysiology. Clinically, patients may be completely asymptomatic and the diagnosis is only made incidentally through imaging tests. The discovery could be made when complications occur such as intraperitoneal hemorrhage with hypovolemic shock due to the rupture of an adenoma. We report a fatal case of a ruptured adenoma in a case of hepatic adenomatosis discovered at autopsy. In order to achieve a better view of this disease, we conducted a literature review on this subject describing the pathogenesis, manifestations, and autopsy contribution to addressing this entity.
摘要:
肝腺瘤病是一种罕见的疾病,由原本正常的肝实质中的多发性腺瘤组成。虽然这个实体的发现可以追溯到几年前,就其定义和病理生理学而言,其诊断仍然具有挑战性。临床上,患者可能完全无症状,仅通过影像学检查进行诊断。当发生并发症时,例如由于腺瘤破裂引起的腹膜内出血伴低血容量性休克,可以发现。我们报告了在尸检中发现的一例肝腺瘤病中腺瘤破裂的致命病例。为了更好地了解这种疾病,我们对这一主题进行了文献综述,描述了发病机理,表现,和尸检有助于解决这个实体。