Liver transplantation for adenomatosis: European experience

Abstract

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.

Transplantation as disease modifying therapy in adults with inherited metabolic disorders

“…While documented hepatocellular carcinoma qualifies for a MELD exemption, adenomas do not although, in GSDs, there is no good noninvasive way (other than growth) to tell if an adenoma has undergone malignant transformation as tumor markers are usually negative (Kishnani et al 2014). Fortunately, a recent review of transplantation for adenomas in Europe suggests that the actual risk of hepatocellular carcinoma in adenomas was lower than previously thought (one case out of 33 patients in whom hepatocellular carcinoma was not diagnosed preoperatively based on imaging or biopsy) and did not differ between patients with GSD from those with adenomas for other reasons (Chiche et al 2016).…”

2018 Annual Report of the European Liver Transplant Registry (ELTR) - 50-year evolution of liver transplantation

“…A sample of these studies is cited in the references of the manuscript. With reports concerning LT for specific hepatic diseases [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24], analysis of the impact of the type of preservation solution [25], and of the immunosuppressive regimen on the patient outcome [26], ELTR has helped develop risk models for mortality following liver-transplantation [27,28]. Owing to the large cohort of patients, the exhaustiveness, and quality of the data, and the long follow-up provided by the ELTR, the results are really representative of LT in Europe.…”

Challenging Issues in Hepatic Adenoma

“…Transplanted liver is generally harvested from a cadaveric donor but living liver transplantation has also been reported [71]. Due to an expanding armamentarium and experience in angiographically controlling bleeding from a ruptured HA, liver transplantation as an ultimate life-rescue therapy remains exceptionally rare, being reported for spontaneous intra-partum rupture of hepatocellular adenoma [72] (Algorithm 1).…”

“…Liver transplantation should be considered as the last resort for patients with adenomatosis. Patients with GSD should undergo transplantation earlier than other patients with HA because the literature considers this underlying disease as a risk factor for malignant transformation of adenomas [72]. Like in transplantation for HCC, imaging diagnosis of vascular invasion should be considered an absolute contraindication to transplantation.…”

“…The only major criterion is the histological proof of malignancy in at least one adenoma. The minor criteria are: (1) more than 2 serious (life-threatening) hemorrhages, (2) more than 2 previous hepatectomies, (3) β mutated or inflammatory adenomas, (4) underlying liver disease (major steatosis and vascular abnormalities), and (5) age > 30 years [72] (Figure 20).…”