Gastric neuroendocrine neoplasm with late liver metastasis.

Gastric neuroendocrine neoplasms (GNENs) are classified into three types according to their aetiology. We present a clinical case of a female patient of 66 years and a well-differentiated (grade 2), type 3 GNEN with late liver metastasis (LM). The patient underwent surgical excision of a gastric lesion at 50 years of age, without any type of follow-up. Sixteen years later, she was found to have a neuroendocrine tumour (NET) metastatic to the liver. The histological review of the gastric lesion previously removed confirmed that it was a NET measuring 8 mm, pT1NxMx (Ki67 = 4%). 68Ga-DOTANOC PET/CT reported two LM and a possible pancreatic tumour/gastric adenopathy. Biopsies of the lesion were repeatedly inconclusive. She had a high chromogranin A, normal gastrin levels and negative anti-parietal cell and intrinsic factor antibodies, which is suggestive of type 3 GNEN. She underwent total gastrectomy and liver segmentectomies (segment IV and VII) with proven metastasis in two perigastric lymph nodes and both with hepatic lesions (Ki67 = 5%), yet no evidence of local recurrence. A 68Ga-DOTANOC PET/CT was performed 3 months after surgery, showing no tumour lesions and normalisation of CgA. Two years after surgery, the patient had no evidence of disease. This case illustrates a rare situation, being a type 3, well-differentiated (grade 2) GNEN, with late LM. Despite this, it was possible to perform surgery with curative intent, which is crucial in these cases, as systemic therapies have limited efficacy. We emphasise the need for extended follow-up in these patients. LEARNING POINTS: GNENs have a very heterogeneous biological behaviour.Clinical distinction between the three types of GNEN is essential to plan the correct management strategy.LMs are rare and more common in type 3 and grade 3 GNEN.Adequate follow-up is crucial for detection of disease recurrence.Curative intent surgery is the optimal therapy for patients with limited and resectable LM, especially in well-differentiated tumours (grade 1 and 2).

Auxiliary Liver Transplantation as a Transient Treatment for Acute Liver Failure: Two Cases.

INTRODUCTION: Acute liver failure is an uncommon condition associated with a high mortality. Most patients do not survive without liver transplantation. In the last decades, auxiliary liver transplantation has emerged as a therapeutic option. CLINICAL CASE: The authors present two cases of acute liver failure that required liver transplantation. Given the patients' young age and the preserved macroscopic liver pattern evaluated in surgery, auxiliary liver transplantation was executed using different surgical approaches. Afterwards, following confirmed full native liver regeneration, the patients were submitted to auxiliary liver hepatectomy, which was accomplished without complications. CONCLUSION: Auxiliary liver transplantation can be regarded as an effective temporary treatment for acute liver failure in selected cases, allowing an immunosuppression-free life.

INTRODUÇÃO: A falência hepática aguda é uma entidade clínica pouco comum, mas associada a elevada mortalidade. A maioria dos doentes não sobreviverá sem transplante hepático. Nas últimas décadas, o transplante hepático auxiliar tem sido utilizado como uma opção terapêutica valorizável. CASO CLÍNICO: Apresentam-se dois casos de falência hepática aguda tratados com transplante hepático. Tendo em conta a idade jovem dos doentes e a noção de preservação macroscópica do fígado, recorreu-se à opção de transplante hepático auxiliar utilizando técnicas diferentes. Posteriormente, após confirmação de regeneração hepática completa, procedeu-se à hepatectomia do fígado auxiliar. CONCLUSÃO: O transplante hepático auxiliar constitui uma terapêutica transitória eficaz em alguns casos de falência hepática aguda, permitindo um futuro isento de imunossupressão.

Groove Pancreatitis with Biliary and Duodenal Stricture: An Unusual Cause of Obstructive Jaundice.

INTRODUCTION: Groove pancreatitis is an uncommon cause of chronic pancreatitis that affects the groove anatomical area between the head of the pancreas, duodenum, and common bile duct. CLINICAL CASE: A 67-year-old man with frequent biliary colic and an alcohol consumption of 30-40 g/day was admitted to the hospital complaining of jaundice and pruritus. Laboratory analysis revealed cholestasis and the ultrasound scan showed intra-hepatic biliary ducts dilatation, middle third cystic dilatation of common bile duct, enlarged Wirsung and pancreatic atrophy. The magnetic resonance cholangiopancreatography showed imaging findings compatible with groove pancreatitis. An esophagogastroduodenoscopy later excluded duodenal neoplasia. He was submitted to a Roux-en-Y cholangiojejunostomy because of common bile duct stricture. Five months later a gastrojejunostomy was performed due to a duodenal stricture. The patient remains asymptomatic during follow-up. DISCUSSION: Groove pancreatitis is a benign cause of obstructive jaundice, whose main differential diagnosis is duodenal or pancreatic neoplasia. When this condition causes duodenal or biliary stricture, surgical treatment can be necessary.

INTRODUÇÃO: A pancreatite da goteira duodeno-pancreática é uma forma rara de pancreatite crónica, que afeta a área anatómica entre a cabeça do pâncreas, duodeno e ducto biliar comum. CASO CLÍNICO: Doente do sexo masculino, 67 anos, com antecedentes de cólicas biliares de repetição e consumo etílico de 30-40 g/dia, internado por icterícia e prurido. Analiticamente, apresentava colestase e, ecograficamente, dilatação moderada das vias biliares intra-hepáticas (VBIH), dilatação quística do 1/3 médio do colédoco, ectasia do Wirsung e atrofia pancreática. A colangiopancreatografia por ressonância demonstrou aspetos imagiológicos compatíveis com pancreatite paraduodenal. A endoscopia alta excluiu neoplasia duodenal. Foi submetido a colangiojejunostomia em Y Roux por estenose do colédoco e após 5 meses a gastrojejunostomia por estenose duodenal. O doente mantem seguimento, permanecendo assintomático. DISCUSSÃO: A pancreatite paraduodenal é uma forma benigna de icterícia obstrutiva, cujo principal diagnóstico diferencial é a neoplasia duodenal/pancreática. Quando esta condição causa estenose duodenal ou biliar, a terapêutica cirúrgica poderá ser necessária.

Gene modulation associated with inhibition of liver regeneration in hepatitis B virus X transgenic mice.

AIM: To analyze the modulation of gene expression profile associated with inhibition of liver regeneration in hepatitis B X (HBx)-expressing transgenic mice. METHODS: Microarray technology was performed on liver tissue obtained from 4 control (LacZ) and 4 transgenic mice (HBx-LacZ), 48 h after partial hepatectomy. The significance of the normalized log-ratios was assessed for each gene, using robust t-tests under an empirical Bayes approach. Microarray hybridization data was verified on selected genes by quantitative PCR. RESULTS: The comparison of gene expression patterns showed a consistent modulation of the expression of 26 genes, most of which are implicated in liver regeneration. Up-regulated genes included DNA repair proteins (Rad-52, MSH6) and transmembrane proteins (syndecan 4, tetraspanin), while down-regulated genes were connected to the regulation of transcription (histone deacetylase, Zfp90, MyoD1) and were involved in the cholesterol metabolic pathway and isoprenoid biosynthesis (farnesyl diphosphate synthase, Cyp7b1, geranylgeranyl diphosphate synthase, SAA3). CONCLUSION: Our results provide a novel insight into the biological activities of HBx, implicated in the inhibition of liver regeneration.

[Adult nesidioblastosis]. / Nesidioblastose do adulto.

In adults, endogenous hyperinsulinemic hypoglycaemia is almost invariably due to insulinoma. The authors describe a 29 year old patient with repeated postprandial symptomatic hypoglycaemias and negative prolonged fast test. Distal pancreatectomy was performed, directed by positive arteriographic testing. Histological examination revealed pancreatic nesidioblastosis and no tumoral lesion. Distinct pathogenetic, clinical, diagnostic and therapeutic aspects of this rare disease in the adult are reviewed.