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Intrahepatic cholangiocarcinoma (ICC) is the second most common primary hepatic malignant tumor and its incidence is increasing over the world. At present times, radical liver resection is still the most effective treatment for ICC patients to achieve long term survival. Pathological lymph node metastases (LMN), found in 15% to 45% of the patients, have been recognized as an extremely poor prognostic risk factor, even if curative resection is performed. So, considering this issue, it acquires relevance to determine the validity of surgical resection for LNM cases that are diagnosed in the preoperative setting, or whether a routine lymphadenectomy should be performed systematically in all hepatectomies for ICC. The role of routine lymphadenectomy in the surgical treatment of ICC remains controversial, with some centers considering it standard whereas other surgeons perform lymphadenectomy only as a selective indication. Recently, a growing widespread adoption of lymphadenectomy was demonstrated that nearly doubled its commonly reported execution rate. The newly updated eight edition of the American Joint Committee on Cancer (AJCC) staging system now recommends that six nodes need to be analyzed to stage patients with ICC. In this review, we analyzed and summarized some anatomic considerations of the lymphatic anatomy of the liver and the current knowledge and potential advantages of performing a routine lymphadenectomy in patients with ICC, especially looking at pathological staging, prognosis, prevention of local recurrence and outcome. New areas like lymphadenectomy in cirrhotic patients and laparoscopic lymphadenectomy are also discussed.

In July 2005, Argentina became the first country after the United States to introduce the Model for End-Stage Liver Disease (MELD) for organ allocation. In this study, we investigated waiting-list (WL) outcomes (n = 3272) and post-liver transplantation (LT) survival in 2 consecutive periods of 5 years before and after the implementation of a MELD-based allocation policy. Data were obtained from the database of the national institute for organ allocation in Argentina. After the adoption of the MELD system, there were significant reductions in WL mortality [28.5% versus 21.9%, P < 0.001, hazard ratio (HR) = 1.57, 95% confidence interval (CI) = 1.37-1.81] and total dropout rates (38.6% versus 29.1%, P < 0.001, HR = 1.31, 95% CI = 1.16-1.48) despite significantly less LT accessibility (57.4% versus 50.7%, P < 0.001, HR = 1.53, 95% CI = 1.39-1.68). The annual number of deaths per 1000 patient-years at risk decreased from 273 in 2005 to 173 in 2010, and the number of LT procedures per 1000 patient-years at risk decreased from 564 to 422. MELD and Model for End-Stage Liver Disease-Sodium scores were excellent predictors of 3-month WL mortality with c statistics of 0.828 and 0.857, respectively (P < 0.001). No difference was observed in 1-year posttransplant survival between the 2 periods (81.1% versus 81.3%). Although patients with a MELD score > 30 had lower posttransplant survival, the global accuracy of the score for predicting outcomes was poor, as indicated by a c statistic of only 0.523. Patients with granted MELD exceptions (158 for hepatocellular carcinoma and 52 for other reasons) had significantly higher access to LT (80.4%) in comparison with nonexception patients with equivalent listing priority (MELD score = 18-25; 54.6%, P < 0.001, HR = 0.49, 95% CI = 0.40-0.61). In conclusion, the adoption of the MELD model in Argentina has resulted in improved liver organ allocation without compromising posttransplant survival., (© 2013 American Association for the Study of Liver Diseases.)

Objectives: In patients diagnosed with incidental gallbladder cancer (GC), the benefit and optimal extent of further surgery remain unclear. The aims of this study were to analyse outcomes in patients who underwent liver resection following a diagnosis of incidental GC and to determine factors associated with longterm survival., Methods: A retrospective analysis of patients diagnosed with incidental GC between June 1999 and June 2010 was performed. Data covering demographics, clinical and surgical characteristics and local pathological stage were analysed., Results: A total of 24 patients were identified. All patients underwent a resection of segments IVb and V and lymphadenectomy. Histological examination revealed residual disease in 10 patients, all of whom presented with recurrent disease at 3-12 months. Overall 5-year survival was 53%. Increasing T-stage (P < 0.001), tumour-node-metastasis (TNM) stage (P= 0.003), and the presence of residual tumour in the resected liver (P < 0.001) were all associated with worse survival., Conclusions: Aggressive re-resection of incidental GC offers the only chance for cure, but its efficacy depends on the extent of disease found at the time of repeat surgery. The presence of residual disease correlated strongly with T-stage and was the most relevant prognostic factor for survival in patients treated with curative resection., (© 2012 International Hepato-Pancreato-Biliary Association.)

Background: Caroli's disease (CD) management is still controversial., Aim: The purpose of this study is to report the most frequent clinical features, treatment options, and outcome obtained after surgical management of CD., Methods: A voluntary survey was conducted. Demographic, clinical, surgical, and pathological variables were analyzed., Results: Six centers included 24 patients having received surgical treatment from 1991 to 2009. Seventeen (70.8%) patients were female, with average age of 48.7 years old (20-71), and 95.5% were symptomatic. There was left hemiliver involvement in 75% of the patients. Surgical procedures included nine left lateral sectionectomies, eight left hepatectomies, and four right hepatectomies for those with hemiliver disease, while for patients with bilateral disease, one right hepatectomy and two Roux-en-Y hepaticojejunostomies were performed. The average length of hospitalization was 7 days. For perioperative complications (25%), three patients presented minor complications (types 1-2), while major complications occurred in three patients (type 3a). No mortality was reported. After a median follow-up of 166 months, all patients are alive and free of symptoms. CD diagnosis was confirmed by histology. Congenital hepatic fibrosis was present in two patients (8.3%) and cholangiocarcinoma in one (4.2%)., Conclusions: CD in Argentina is more common in females with left hemiliver involvement. Surgical resection is the best curative option in unilateral disease, providing long-term survival free of symptoms and complications. In selected cases of bilateral disease without parenchymal involvement, hepaticojejunostomy should be proposed. However, a close follow-up is mandatory because patients might progress and a transplant should be indicated.

Objective: To evaluate the oxidative state in patients with familial amyloidotic polyneuropathy type 1 (FAP1)., Design: From 3 unrelated families, patients with FAP1 carrying a transthyretin Met-30 mutation were studied. The diagnosis was confirmed by genetic analysis. Eleven of 21 patients carried the mutation; all were symptomatic and were clinically assessed using a clinical score. All of the patients were evaluated for copper-zinc superoxide dismutase type 1 activity in red blood cells using spectrophotometry. Plasma total reactive antioxidant potential was studied using a chemiluminescent method. The results were compared with those obtained from an age-matched control group., Setting: A public and academic multidisciplinary research clinic., Results: Six of the 11 FAP1-positive patients disclosed superoxide dismutase type 1 activity values greater than 55 U/mg of protein (upper control limit), whereas 9 of 10 patients in whom total reactive antioxidant potential was measured had values below the lower limit of the control group. No relationship was found between the levels of superoxide dismutase type 1 activity and the severity of the clinical involvement., Conclusions: Oxidative stress may be part of the mechanisms leading to tissue damage in patients with FAP1. The lack of correlation between the laboratory findings and the severity of clinical involvement may signal that oxidative processes are at work throughout the natural history of the disease.

We have developed a quick and reliable diagnostic method for detecting variant forms of transthyretin (TTR); namely, centrifugal concentration followed by electrospray ionization mass spectrometry (ESI-MS). Argentinian patients from three families with neuropathic amyloidosis and their relatives were screened for mutated TTR by ESI-MS. In order to facilitate transportation, we investigated the impact storage had on lyophilized anti-TTR-antibody precipitates' mass spectra. For this investigation, plasma samples from three Swedish patients with known TTR amyloidosis were analysed. We detected identical, additional peaks corresponding to a variant form of TTR in 10 members of the families, and also in a lyophilized sample sent unfrozen by mail from Argentina. All except one symptomatic subject had additional peaks, the exception having undergone a liver transplantation for the disease. All patients were early onset cases, i.e. below 35 years of age, and family history suggests an aggressive, rapidly progressing disease. Lyophilized anti-TTR-antibody precipitates stored at room temperature for 1 week exhibited only minor differences compared with plasma samples stored at -70 degrees C. In a new Argentinian study on familial amyloidotic polyneuropathy, the variant TTR was quickly identified and typed by ESI-MS. To facilitate transportation, dry-frozen samples can be used and the quality of the spectra is similar to that of samples stored at -70 degrees C.