脂肪瘤症是脂肪组织的良性增生。脂肪瘤(良性脂肪瘤)是脂肪瘤病的最常见成分。它们可能是独一无二的,也可能是多重的,封装或不封装,皮下或有时内脏。在某些情况下,它们形成大面积的非包裹脂肪肥大,纤维化程度不同。尽管没有肥胖,它们仍然可以发展。它们可能是家族性的,也可能是后天获得的。与脂肪营养不良综合征不同,它们与脂肪萎缩区域无关,除了一些罕见的病例,如2型家族性部分脂肪营养不良综合征(FPLD2)。它们的代谢影响是可变的,部分取决于相关的肥胖。它们可能具有功能或美学后果。脂肪瘤病可能是孤立的,是综合症的一部分,或者可能是内脏的。孤立性脂肪瘤病包括多发性对称性脂肪瘤病(Madelung病或Launois-Bensaude综合征),家族性多发性脂肪瘤病,痛苦的皮肤病也被称为肥胖Dolorosa或Ander综合征,间质脂肪瘤病也称为Roch-Leri脂肪瘤病,家族性血管脂肪瘤病,lipedema和hibernomas.综合征性脂肪瘤病包括PIK3CA相关疾病,Cowden/PTEN错构瘤-肿瘤综合征,一些脂肪营养不良综合征,和线粒体疾病,尤其是MERRF,多发性内分泌瘤1型,神经纤维瘤病1型,威尔逊病,Pai或Haberland综合征。最后,内脏脂肪瘤在许多器官和部位都有报道:胰腺,肾上腺,腹部,硬膜外,纵隔,本综述的目的是介绍脂肪瘤病的主要类型及其病理生理成分。当它是已知的。
Lipomatoses are benign proliferation of adipose tissue. Lipomas (benign fat tumors) are the most common component of lipomatosis. They may be unique or multiple, encapsulated or not, subcutaneous or sometimes visceral. In some cases, they form large areas of non-encapsulated fat hypertrophy, with a variable degree of fibrosis. They can develop despite the absence of obesity. They may be familial or acquired. At difference with lipodystrophy syndromes, they are not associated with lipoatrophy areas, except in some rare cases such as type 2 familial partial lipodystrophy syndromes (FPLD2). Their metabolic impact is variable in part depending on associated obesity. They may have functional or aesthetic consequences. Lipomatosis may be isolated, be part of a syndrome, or may be visceral. Isolated lipomatoses include multiple symmetrical lipomatosis (Madelung disease or Launois-Bensaude syndrome), familial multiple lipomatosis, the painful Dercum\'s disease also called Adiposis Dolorosa or Ander syndrome, mesosomatic lipomatosis also called Roch-Leri lipomatosis, familial angiolipomatosis, lipedema and hibernomas. Syndromic lipomatoses include PIK3CA-related disorders, Cowden/PTEN hamartomas-tumor syndrome, some lipodystrophy syndromes, and mitochondrial diseases, especially MERRF, multiple endocrine neoplasia type 1, neurofibromatosis type 1, Wilson disease, Pai or Haberland syndromes. Finally, visceral lipomatoses have been reported in numerous organs and sites: pancreatic, adrenal, abdominal, epidural, mediastinal, epicardial… The aim of this review is to present the main types of lipomatosis and their physiopathological component, when it is known.