Usefulness of PIVKA-II for monitoring after liver transplantation in patients with hepatocellular carcinoma.

The high morbidity and mortality of hepatocellular carcinoma (HCC) has encouraged the search for new biomarkers to be used alongside alpha-foetoprotein (AFP) and imaging tests. The aim of this study was to evaluate the clinical contribution of protein induced by vitamin K absence or antagonist-II (PIVKA-II) for HCC monitoring after liver transplantation (LT) and compare it with AFP, a routinely used tumour marker. A total of 46 HCC patients (Milan criteria) were enrolled in this study. Serum levels of PIVKA-II and AFP were measured before and after transplantation. Clinical features were determined for all the patients that were included. Significant correlations were found between PIVKA-II expression levels and some clinicopathological features, such as tumour size and number of pre-transplant transarterial chemoembolizations (TACEs). Serum levels of PIVKA-II and AFP decreased significantly after LT and increased in patients with tumour recurrence. Serum PIVKA-II levels may play an important role in predicting disease severity. Furthermore, monitoring PIVKA-II levels in HCC transplant recipients reflects the tumor early recurrence after transplantation and could be used, complementing AFP and imaging tests, as a novel biomarker of this pathology.

Functional Characterisation of Bile Metagenome: Study of Metagenomic Dark Matter.

Gallbladder metagenome involves a wide range of unidentified sequences comprising the so-called metagenomic dark matter. Therefore, this study aimed to characterise three gallbladder metagenomes and a fosmid library with an emphasis on metagenomic dark matter fraction. For this purpose, a novel data analysis strategy based on the combination of remote homology and molecular modelling has been proposed. According to the results obtained, several protein functional domains were annotated in the metagenomic dark matter fraction including acetyltransferases, outer membrane transporter proteins, membrane assembly factors, DNA repair and recombination proteins and response regulator phosphatases. In addition, one deacetylase involved in mycothiol biosynthesis was found in the metagenomic dark matter fraction of the fosmid library. This enzyme may exert a protective effect in Actinobacteria against bile components exposure, in agreement with the presence of multiple antibiotic and multidrug resistance genes. Potential mechanisms of action of this novel deacetylase were elucidated by molecular simulations, highlighting the role of histidine and aspartic acid residues. Computational pipelines presented in this work may be of special interest to discover novel microbial enzymes which had not been previously characterised.

Intraductal papillary mucinous neoplasm: a new entity among biliary tract neoplasms.

Biliary tract intraductal papillary mucinous neoplasms (BT-IPMN) are a rare entity that develop within the bile duct lumen and are recognized as a precursor of invasive carcinoma in up to 40-80 % of cases.

Hepatic epithelioid hemangioendothelioma: a rare vascular tumor with an unpredictable diagnosis.

We present the case of a 58-year-old female with no relevant medical or surgical history, who was referred to our hospital with a solitary hepatic lesion. She presented a slightly abnormal liver function in a routine blood test: ALT 71 U/l (range 0-33), AST 40 U/l (range 0-32) and GGT 71 U/l (range 0-40), with no symptoms. Ultrasound imaging showed a 3-cm-size focal lesion with a peripheral hypoechoic rim in the right hepatic lobe. Abdominal computed tomography (CT) scan after contrast injection revealed a subcapsular hypodense lesion in segment VII with capsular retraction. Other extrahepatic lesions were not seen.

Ventral Portal Vein Over Gallbladder and Pancreas---An Exceptional Anatomical Variation Found in a Liver Transplant Patient: A Case Report.

BACKGROUND: Anatomic variations are well known in the liver hilum. A rare precholecystic, preduodenal, prepancreatic portal vein is described as found in a liver transplant candidate. Precholecystic location of portal vein is an exceptional finding and does not seem to have been previously described. It is associated with a preduodenal portal vein. Its position is challenging, as its surface can be mistaken with the gallbladder wall. We present the case of a patient candidate to liver transplantation. In the preoperative studies, a portal thrombosis was suspected, with recanalization by collaterals, but also a malformation was suggested. The patient had a primary biliary cirrhosis. Other findings included agenesis of inferior vena cava on the right side. During operation, the portal vein was found over the gallbladder and fixed to it, making it at first difficult to distinguish one from the other. CONCLUSIONS: A precholecystic portal vein is a rare finding that poses a challenge for the surgeon. It must be ruled out in the preoperative workout.

The human gallbladder microbiome is related to the physiological state and the biliary metabolic profile.

BACKGROUND: The microbial populations of the human intestinal tract and their relationship to specific diseases have been extensively studied during the last decade. However, the characterization of the human bile microbiota as a whole has been hampered by difficulties in accessing biological samples and the lack of adequate methodologies to assess molecular studies. Although a few reports have described the biliary microbiota in some hepatobiliary diseases, the bile microbiota of healthy individuals has not been described. With this in mind, the goal of the present study was to generate fundamental knowledge on the composition and activity of the human bile microbiota, as well as establishing its potential relationship with human bile-related disorders. RESULTS: Human bile samples from the gallbladder of individuals from a control group, without any record of hepatobiliary disorder, were obtained from liver donors during liver transplantation surgery. A bile DNA extraction method was optimized together with a quantitative PCR (qPCR) assay for determining the bacterial load. This allows the selection of samples to perform functional metagenomic analysis. Bile samples from the gallbladder of individuals suffering from lithiasis were collected during gallbladder resection and the microbial profiles assessed, using a 16S rRNA gene-based sequencing analysis, and compared with those of the control group. Additionally, the metabolic profile of the samples was analyzed by nuclear magnetic resonance (NMR). We detected, for the first time, bacterial communities in gallbladder samples of individuals without any hepatobiliary pathology. In the biliary microecosystem, the main bacterial phyla were represented by Firmicutes, Bacteroidetes, Actinobacteria, and Proteobacteria. Significant differences in the relative abundance of different taxa of both groups were found. Sequences belonging to the family Propionibacteriaceae were more abundant in bile samples from control subjects; meanwhile, in patients with cholelithiasis members of the families Bacteroidaceae, Prevotellaceae, Porphyromonadaceae, and Veillonellaceae were more frequently detected. Furthermore, the metabolomics analysis showed that the two study groups have different metabolic profiles. CONCLUSIONS: Our results indicate that the gallbladder of human individuals, without diagnosed hepatobiliary pathology, harbors a microbial ecosystem that is described for the first time in this study. Its bacterial representatives and metabolites are different from those detected in people suffering from cholelithiasis. In this regard, since liver donors have been subjected to the specific conditions of the hospital's intensive care unit, including an antibiotic treatment, we must be cautious in stating that their bile samples contain a physiologically normal biliary microbiome. In any case, our results open up new possibilities to discover bacterial functions in a microbial ecosystem that has not previously been explored.

Epigenetic Deregulation of Protocadherin PCDHGC3 in Pheochromocytomas/Paragangliomas Associated With SDHB Mutations.

CONTEXT: SDHB mutations are found in an increasing number of neoplasms, most notably in paragangliomas and pheochromocytomas (PPGLs). SDHB-PPGLs are slow-growing tumors, but ∼50% of them may develop metastasis. The molecular basis of metastasis in these tumors is a long-standing and unresolved problem. Thus, a better understanding of the biology of metastasis is needed. OBJECTIVE: This study aimed to identify gene methylation changes relevant for metastatic SDHB-PPGLs. DESIGN: We performed genome-wide profiling of DNA methylation in diverse clinical and genetic PPGL subtypes, and validated protocadherin γ-C3 (PCDHGC3) gene promoter methylation in metastatic SDHB-PPGLs. RESULTS: We define an epigenetic landscape specific for metastatic SDHB-PPGLs. DNA methylation levels were found significantly higher in metastatic SDHB-PPGLs than in SDHB-PPGLs without metastases. One such change included long-range de novo methylation of the PCDHA, PCDHB, and PCDHG gene clusters. High levels of PCDHGC3 promoter methylation were validated in primary metastatic SDHB-PPGLs, it was found amplified in the corresponding metastases, and it was significantly correlated with PCDHGC3 reduced expression. Interestingly, this epigenetic alteration could be detected in primary tumors that developed metastasis several years later. We also show that PCDHGC3 down regulation engages metastasis-initiating capabilities by promoting cell proliferation, migration, and invasion. CONCLUSIONS: Our data provide a map of the DNA methylome episignature specific to an SDHB-mutated cancer and establish PCDHGC3 as a putative suppressor gene and a potential biomarker to identify patients with SDHB-mutated cancer at high risk of metastasis who might benefit from future targeted therapies.

Villous adenoma of the choledocho: an infrequent pathology of the main bile duct.

We present the case of a patient with obstructive jaundice due to a polylobulated lesion at the middle common bile duct that is identified as a villous adenoma of the main bile duct. This entity is infrequent with few references in the bibliography. It is a benign pathology but with risk of malignancy, so the diagnosis and resection is essential for adequate treatment.

Rectorragia como forma de presentación de un caso de síndrome de Abernethy en el adulto / Lower gastrointestinal bleeding as a form of presentation in an adult case of Abernethy syndrome

Presentamos el caso de una paciente de 29 años que consultó por rectorragias autolimitadas que fue diagnosticada de síndrome de Abernethy (shunt portosistémico congénito). Se trata de un debut clínico poco habitual en este tipo de síndrome congénito muy poco prevalente. La gran mayoría de los casos descritos en la literatura se manifiestan clínicamente en la edad pediátrica

We report the case of a 29-year-old patient who presented with a short history of lower gastrointestinal bleeding. A diagnosis of Abernethy syndrome was made (congenital extrahepatic portosystemic shunt) after this uncommon clinical presentation. The prevalence of this congenital malformation is very low and usually manifests during the pediatric age, according to previously published reports

Villous adenoma of the choledocho: an infrequent pathology of the main bile duct / Adenoma velloso de colédoco: una patología infrecuente de la vía biliar principal

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Lower gastrointestinal bleeding as a form of presentation in an adult case of Abernethy syndrome.

We report the case of a 29-year-old patient who presented with a short history of lower gastrointestinal bleeding. A diagnosis of Abernethy syndrome was made (congenital extrahepatic portosystemic shunt) after this uncommon clinical presentation. The prevalence of this congenital malformation is very low and usually manifests during the pediatric age, according to previously published reports.

Hemoperitoneo masivo por rotura de vena umbilical en paciente cirrótico / Massive hemoperitoneum due to a ruptured umbilical vein in a cirrhotic patient

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Tumor neuroendocrino primario hepático en paciente con hepatitis aguda VHB: una neoplasia inusual / Primary hepatic neuroendocrine tumor in a patient with acute HBV hepatitis: An unusual neoplasia

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Inflammatory pseudotumor of spleen: a case report.

INTRODUCTION: Inflammatory pseudotumor of spleen is an extremely rare benign condition of uncertain etiology that presents with nonspecific symptoms or as an incidental finding in patients studied by other processes. Since the first description in 1984 by Cotelingam and Jaffe, only 114 cases have been reported. PRESENTATION OF CASE: We present a case of a fifty-six years old woman with a splenic injury in ultrasound and computed tomography. The patient undergoes laparoscopic splenectomy and the histologic study of the specimen revealed findings consistent with inflammatory pseudotumor of spleen. DISCUSSION: This rare entity whose pathogenesis is still unknown, can present with nonspecific symptoms. Radiologic studies may lead the diagnosis being useful CT and MRI. The definitive diagnosis is established with the histological findings, characterized by the presence of inflammatory cells with areas of necrosis and fibrosis. There are multiple differentials diagnoses: metastasis, lymphoma, splenic infarction, hemangiomas, vascular malformations, lymphangioma, plasmacytoma, reactive lymphoid hyperplasia, abscess and infectious granulomatous processes; therefore suspicion of malignant neoplasm must be considered, being indicated splenectomy to confirm the diagnosis. CONCLUSION: Inflammatory pseudotumor of spleen is a benign disease, in which diagnostic approach must bear in mind the possibility of a malignant lesion. For this reason, the surgical approach is appropriate to confirm the diagnosis and rule out malignancy with histology.

Estudio multicéntrico sobre adenomas hepáticos / Multicentre study on hepatic adenomas

INTRODUCCIÓN: El adenoma hepático (AH) es un tumor benigno que puede presentar graves complicaciones por lo que, clásicamente, todos eran resecados. Actualmente se ha demostrado que los menores de 3 cm, y si no expresan Beta -catenina, solo se complican excepcionalmente, lo que ha cambiado la estrategia terapéutica. MATERIAL Y MÉTODOS: Estudio retrospectivo en 14 unidades HPB. Criterio de inclusión: pacientes con AH resecado y confirmado histológicamente. Periodo de estudio: 1995-2011. RESULTADOS: Fueron intervenidos 81 pacientes. Edad: 39,5 años (rango: 14-75). Sexo: mujeres (75%). Consumo de estrógenos en mujeres: 33%. Tamaño: 8,8 cm (rango: 1-20 cm). Solo 6 AH (7,4%) eran menores de 3 cm. La mediana de AH fue 1 (rango: 1-12). Nueve pacientes presentaban adenomatosis (> 10 AH). El 51% de los pacientes presentaban síntomas; el más frecuente (77%) era dolor abdominal. Ocho pacientes (10%) comenzaron con abdomen agudo por rotura o hemorragia. El 67% de los diagnósticos preoperatorios fueron correctos. La cirugía fue programada en el 90% de los pacientes. Las técnicas fueron: hepatectomías mayores (22%), menores (77%) y un trasplante hepático. Un 20% fueron realizadas por laparoscopia. La morbilidad fue 28%. No hubo mortalidad. Tres pacientes presentaron malignización (3,7%). El seguimiento fue 43 meses (rango: 1-192). Se detectaron 2 recidivas que fueron resecadas. DISCUSIÓN: Los pacientes con AH resecados son habitualmente mujeres con lesiones grandes, con un consumo de estrógenos inferior al esperado. Su diagnóstico preoperatorio correcto es aceptable (70%). La tasa de hepatectomías mayores es 25% y la de laparoscopia, 20%. Hemos obtenido una baja morbilidad y nula mortalidad

INTRODUCTION: Hepatic adenomas (HA) are benign tumours which can present serious complications, and as such, in the past all were resected. It has now been shown that those smaller than 3 cm not expressing Beta-catenin only result in complications in exceptional cases and therefore the therapeutic strategy has been changed. MATERIAL AND METHOD: Retrospective study in 14 HPB units. Inclusion criteria: patients with resected and histologically confirmed HA. Study period: 1995-2011. RESULTS: 81 patients underwent surgery. Age: 39.5 years (range: 14-75). Sex: female (75%). Consumption of oestrogen in women: 33%. Size: 8.8 cm (range, 1-20 cm). Only 6 HA (7.4%) were smaller than 3 cm. The HA median was 1 (range: 1-12). Nine patients had adenomatosis (> 10HA). A total of 51% of patients displayed symptoms, the most frequent (77%) being abdominal pain. Eight patients (10%) began with acute abdomen due to rupture and/or haemorrhage. A total of 67% of the preoperative diagnoses were correct. Surgery was scheduled for 90% of patients. The techniques employed were: major hepatectomy (22%), minor hepatectomy (77%) and one liver transplantation. A total of 20% were performed laparoscopically. The morbidity rate was 28%. There were no cases of mortality. Three patients had malignisation (3.7%). The follow-up period was 43 months (range 1-192). Two recurrences were detected and resected. DISCUSSION: Patients with resected HA are normally women with large lesions and oestrogen consumption was lower than expected. Its correct preoperative diagnosis is acceptable (70%). The major hepatectomy rate is 25% and the laparoscopy rate is 20%. There was a low morbidity rate and no mortality

Imagen de carcinomatosis peritoneal secundaria a rotura de carcinoma hepatocelular originado sobre hígado sano / Peritoneal carcinomatosis secondary to spread of hepatocellular carcinoma originated upon a healthy liver

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[Multicentre study on hepatic adenomas]. / Estudio multicéntrico sobre adenomas hepáticos.

INTRODUCTION: Hepatic adenomas (HA) are benign tumours which can present serious complications, and as such, in the past all were resected. It has now been shown that those smaller than 3 cm not expressing ß-catenin only result in complications in exceptional cases and therefore the therapeutic strategy has been changed. MATERIAL AND METHOD: Retrospective study in 14 HPB units. INCLUSION CRITERIA: patients with resected and histologically confirmed HA. STUDY PERIOD: 1995-2011. RESULTS: 81 patients underwent surgery. Age: 39.5 years (range: 14-75). Sex: female (75%). Consumption of oestrogen in women: 33%. Size: 8.8 cm (range, 1-20 cm). Only 6 HA (7.4%) were smaller than 3 cm. The HA median was 1 (range: 1-12). Nine patients had adenomatosis (>10HA). A total of 51% of patients displayed symptoms, the most frequent (77%) being abdominal pain. Eight patients (10%) began with acute abdomen due to rupture and/or haemorrhage. A total of 67% of the preoperative diagnoses were correct. Surgery was scheduled for 90% of patients. The techniques employed were: major hepatectomy (22%), minor hepatectomy (77%) and one liver transplantation. A total of 20% were performed laparoscopically. The morbidity rate was 28%. There were no cases of mortality. Three patients had malignisation (3.7%). The follow-up period was 43 months (range 1-192). Two recurrences were detected and resected. DISCUSSION: Patients with resected HA are normally women with large lesions and oestrogen consumption was lower than expected. Its correct preoperative diagnosis is acceptable (70%). The major hepatectomy rate is 25% and the laparoscopy rate is 20%. There was a low morbidity rate and no mortality.