Chiche Laurence, David Anaelle, Adam René, Oliverius M Martin, Klempnauer Jürgen, Vibert Eric, Colledan Michele, Lerut Jan, Mazzafero V Vincenzo, Di-Sandro Stefano, Laurent Christophe, Scuderi Vincenzo, Suc Bertrand, Troisi Roberto, Bachelier Phillipe, Dumortier Jerôme, Gugenheim Jean, Mabrut Jean-Yves, Gonzalez-Pinto Ignacio, Pruvot François-René, Le-Treut Yves Patrice, Navarro Francis, Ortiz-de-Urbina Jorge, Salamé Ephrem, Spada Marco, Bioulac-Sage Paulette
Chirurgie Hépatobiliaire et Pancréatique, Centre Hospitalier Universitaire Bordeaux, Maison du Haut Lévèque, Bordeaux, France.
ELTR and ELITA Board. www.eltr.org.
Liver Transpl. 2016 Apr;22(4):516-26. doi: 10.1002/lt.24417.
The aim of this study was to collect data from patients who underwent liver transplantation (LT) for adenomatosis; to analyze the symptoms, the characteristics of the disease, and the recipient outcomes; and to better define the role of LT in this rare indication. This retrospective multicenter study, based on data from the European Liver Transplant Registry, encompassed patients who underwent LT for adenomatosis between January 1, 1986, and July 15, 2013, in Europe. Patients with glycogen storage disease (GSD) type IA were not excluded. This study included 49 patients. Sixteen patients had GSD, and 7 had liver vascular abnormalities. The main indications for transplantation were either a suspicion of hepatocellular carcinoma (HCC; 15 patients) or a histologically proven HCC (16 patients), but only 17 had actual malignant transformation (MT) of adenomas. GSD status was similar for the 2 groups, except for age and the presence of HCC on explants (P = 0.030). Three patients with HCC on explant developed recurrence after transplantation. We obtained and studied the pathomolecular characteristics for 23 patients. In conclusion, LT should remain an extremely rare treatment for adenomatosis. Indications for transplantation primarily concern the MT of adenomas. The decision should rely on morphological data and histological evidence of MT. Additional indications should be discussed on a case-by-case basis. In this report, we propose a simplified approach to this decision-making process.
本研究的目的是收集因腺瘤病接受肝移植（LT）患者的数据；分析症状、疾病特征和受者结局；并更好地明确LT在这一罕见适应症中的作用。这项基于欧洲肝移植登记处数据的回顾性多中心研究纳入了1986年1月1日至2013年7月15日期间在欧洲因腺瘤病接受LT的患者。不排除患有I型糖原贮积病（GSD）的患者。本研究包括49例患者。16例患者患有GSD，7例有肝脏血管异常。移植的主要指征为怀疑肝细胞癌（HCC；15例患者）或组织学证实的HCC（16例患者），但只有17例腺瘤发生了实际的恶性转化（MT）。两组的GSD状态相似，但年龄和移植肝上HCC的存在情况除外（P = 0.030）。3例移植肝上有HCC的患者移植后出现复发。我们获取并研究了23例患者的病理分子特征。总之，LT对于腺瘤病应仍然是一种极其罕见的治疗方法。移植指征主要涉及腺瘤的MT。决策应基于MT的形态学数据和组织学证据。其他指征应逐案讨论。在本报告中，我们提出了一种简化的决策过程方法。
