Long-term survival after Ltx, especially for early-onset TTR Val30Met patients, is excellent. The risk of delaying Ltx by testing alternative treatments, especially in early-onset TTR Val30Met patients, requires consideration.

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The management of patients with colo-rectal liver metastases has improved significantly over the recent years. However, most patients still cannot undergo complete resection, generally because the location of the metastases within the liver prevents any radical management, and explaining the interest for transplantation. The first attempts were performed in the eighties with poor outcomes, in part due to a high proportion of death not related to the neoplastic disease (1). More recently, the group of Oslo has shown a 60% five-year survival, in 21 patients with colo-rectal metastases (2). Such a survival was better than the one expected on chemotherapy alone (2-4). However, 95% (20/21) of patients had cancer recurrence, most within the first 18 months after transplantation. At present, liver transplantation for colo-rectal metastases remains highly controversial. The potential for long-term disease-free survival needs to be explored, which is the aim of this multi-centric collaborative retrospective study.A total of 12 patients (6 females/6 males) underwent liver transplantation for colo-rectal liver metastasis, at centers affiliated to the "Compagnons Hépato-Bilaires", an association of hepato-pancreato-biliary and transplant surgeons, most trained at the Paul Brousse Hospital, Paris, France under the guidance of Professor H. Bismuth. Median age at transplant was 56 years (Table 1). Patients were managed in Lisbon (n=8), Coimbra (n=2), Paris (n=1), and Geneva (n=1) between October 1995 and October 2015 (date of transplant), and no other patient underwent transplantation for this indication at these respective transplant centers. Data collection was conducted according the relevant ethical standards at each institution. The location of the primary adenocarcinoma was the colon in 11 patients, and the rectum in one. Most primary cancers were T3 on pathology, and many presented between 1 and 3 involved -N1-lymph nodes (two patients were N2 with more than 3 nodes involved, Table 1).For most patients, liver metastases (9/12) were diagnosed within 12 months after the diagnosis of the primary cancer, and were considered as synchronous. When not diagnosed at the same time as the primary, liver metastases were discovered 4, 7, 19, 24 and 29 months after the primary. At the time of transplantation, patients presented a median of 9 liver metastases. Two had lesions >5 cm, of 5.5 and 8 cm. Median CEA level was 16.9 µg/l, and one patients had CEA >200 µg/l, of 314 µg/l.Most (11/12) patients received chemotherapy prior to transplantation. Chemotherapy included irinotecan and oxaliplatin in 9 (82%) patients, and a biological agent in 6 (cetuximab in 2, bevacizumab in 3, and both agents in 1). Another patient was treated by intra-hepatic chemotherapy prior to transplantation. All patients responded to chemotherapy, and none was in progression at the time of the transplantation. The decision to conduct a post-transplant adjuvant chemotherapy was based, at least in the recent years, on the aim to obtain a minimum of four month...

Abstract:Background. This retrospective study was carried out to review our surgical experience and to define the clinicopathologic profile of early gastric cancer in a Western country with one of the highest incidences in the world. Methods. Fifty-eight patients who had gastric surgery for early gastric cancer were included in this study. Results. The incidence of early gastric cancer was 13.9% of the patients with resected gastric cancer (58/416). The mean age of these 58 patients at diagnosis was 56.8 ؎ 12.3 years (range, 30-81 years) and the male : female ratio was 2.4 : 1. The most common presenting symptom was epigastric pain (52.4%). All patients were treated by surgical resection. Tumors were typically located in the antrum (72.4%), with a predominance of lesser curvature lesions (89.7%). Macroscopically, the majority of the lesions (63.8%) were excavated (types IIc and III). Thirty tumors were intramucosal and 28 extended into the submucosa. Thirty were of the intestinal type and 28 of the diffuse type. The rate of regional lymph node metastasis was 10.4%. The overall 5-year survival rate was 93.9%. Conclusions. The excellent response to surgical resection of early gastric cancer reported by Japanese authors is reproducible in Western countries even in the presence of regional lymph node metastasis. For this reason an aggressive surgical approach should be taken for all early gastric cancer.

Background. Patients with liver cirrhosis may develop cirrhotic cardiomyopathy (CC), characterized by blunted contractile responsiveness to stress, diastolic dysfunction (DD), and electrophysiological abnormalities. It may adversely affect the long-term prognosis of these patients. Methods. We conducted a retrospective analysis of patients undergoing liver transplantation (LT) for cirrhosis from January 2012 to June 2015. We analyzed demographic characteristics, the etiology of cirrhosis, Child-Pugh and Model for End-Stage Liver Disease (MELD) scores, the corrected QT (QTc) interval in the preoperative period, diastolic and systolic dysfunction, mortality and survival, and duration of mechanical ventilation and vasopressor support in the post-LT period. These variables were compared with diastolic dysfunction and prolongation of QTc, with the use of chisquare, Fisher, and Mann-Whitney U tests. Results. The study included 106 patients, 80.2% male and overall average age 54.83 years. The median MELD score was 16, and Child-Pugh class C in 55.4%. Prolonged QTc interval before LT was present in 19% and DD in 35.8% of patients. QTc before LT or DD did not vary significantly with MELD or Child-Pugh score. Conclusions. The patients in the pre-LT period presented with a significant incidence of DD, which can predispose them to adverse cardiac events. The presence of DD correlates with mortality after LT in patients with hepatic cirrhosis.

Background: Hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) represent the most common primary liver malignancies whose outcome is influenced by the immune response.Methods: In this study, we have functionally characterized, by flow cytometry, circulating myeloid dendritic cells (mDCs) and FcεRI + monocytes in a group of healthy individuals (n = 10) and in a group of patients with HCC (n = 19) and CCA (n = 8), at the time point of the surgical resection (T0) and once the patient had recovered from surgery (T1). Moreover, we proceeded to a more in depth phenotypic characterization of the FcεRI + monocyte subpopulation.Results: A significant decrease in the frequency of TNFα producing FcεRI + monocytes and mDCs in HCC and CCA patients when compared to the group of healthy individuals was observed, and a close association between FcεRI + monocytes and mDCs dysfunction was identified. In addition, the phenotypic characteristics of FcεRI + monocytes from healthy individuals strongly suggest that this population drives to mDCs, which matches with the fact that both populations are functionally affected.Conclusions: The frequency and the function of circulating mDCs and FcεRI + monocytes are affected in both HCC and CCA patients, and FcεRI + monocytes could represent those fated to become mDCs.

Hepatocellular carcinoma (HCC) in childhood differs from adult HCC because it is often associated with inherited liver disease. It is, however, unclear whether liver transplantation (LT) for HCC in childhood with or without associated inherited disease has a comparable outcome to adult HCC. On the basis of data from the European Liver Transplant Registry (ELTR), we aimed to investigate if there are differences in patient and graft survival after LT for HCC between children and adults and between patients with underlying inherited versus noninherited liver disease, respectively. We included all 175 children who underwent LT for HCC and were enrolled in ELTR between 1985 and 2012. Of these, 38 had an associated inherited liver disease. Adult HCC patients with (n = 79) and without (n = 316, matched by age, sex, and LT date) inherited liver disease served as an adult comparison population. We used multivariable piecewise Cox regression models with shared frailty terms (for LT center) to compare patient and graft survival between the different HCC groups. Survival analyses demonstrated a superior longterm survival of children with inherited liver disease when compared with children with HCC without inherited liver disease (hazard ratio [HR], 0.29; 95% CI, 0.10-0.90; P = 0.03) and adults with HCC with inherited liver disease (HR, 0.27; 95% CI, 0.06-1.25; P = 0.09). There was no survival difference between adults with and without inherited disease (HR, 1.05; 95% CI, 0.66-1.66; P = 0.84). In conclusion, the potential survival advantage of children with an HCC based on inherited disease should be acknowledged when considering transplantation and prioritization for these patients. Further prospective studies accounting for tumor size and extension at LT are necessary to fully interpret our findings. Liver Transplantation 24 246-255 2018 AASLD.

Primary leiomyoma of the liver is an exceptionally rare tumour in non-immunocompromised patients. Preoperative diagnosis of the lesion is difficult as complete imaging of this type of lesion is scarcely defined and preoperative biopsy was not the practice in previously reported cases. We report a voluminous primary leiomyoma of the liver occurring in a healthy middle-aged woman where a preoperative diagnosis was accurately achieved on biopsy. Because of its size, surgery was undertaken for exclusion of malignancy. A 16-month uneventful follow-up has been completed. We discuss the advantage of a preoperative diagnosis and propose that an imaging-guided liver biopsy should be undertaken, provided malignancy features are absent. This could prevent liver surgery merely for diagnostic purposes. Finally, we report imaging features that have not been previously described, namely on magnetic resonance imaging, which may provide an insight about the nature of this particular lesion and, advantageously, contribute toward a non-invasive diagnosis.