Mucinous Cystic Neoplasm of the Liver or Intraductal Papillary Mucinous Neoplasm of the Bile Duct? A Case Report and a Review of Literature.

Mucinous cystic neoplasm of the liver (MCN-L) and intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) are diagnoses that were classified by the World Health Organization in 2010 as mucin-producing bile duct tumors of the hepatobiliary system. The preoperative differential diagnosis between these two entities is difficult; the presence of a communication with the bile duct is usually considered as a typical sign of IPMN-B. However, the presence of an ovarian-like stroma (OLS) has been established to define the diagnosis of MCN-L. We present the case of a 33-year-old woman with a rapid progression of a cystic tumor of the liver. In 2 years, the lesion increased from 27 to 64 mm and a dilation of the left hepatic duct appeared. Percutaneous transhepatic drainage with a biopsy was performed. No malignant cells were found on biopsy. Because of the rapid progression of the cystic tumor and unclear malignant potential, left hemihepatectomy was performed. Even though tumor masses were present in the biliary duct, on the basis of the presence of OLS, histology finally confirmed MCN-L with intermediate-grade intraepithelial dysplasia to high-grade intraepithelial dysplasia. The patient is currently under oncologic follow-up with no signs of recurrence of the disease. We present a rare case where MCN-L caused a dilation of the left hepatic duct, a sign that is usually a characteristic of IPMN-B.

[Mirizzi Syndrome: Prevalence, diagnosis and treatment]. / Síndrome de Mirizzi: Prevalencia, diagnóstico y tratamiento.

BACKGROUND: The Mirizzi syndrome was described by Pablo Luis Mirizzi in 1948, who emphasized this syndrome was characterized by the obstruction of the common hepatic duct due to mechanical compression caused by an impacted stone in the gallbladder neck or in the cystic duct. The incidence ranges from 0.05% to 4%. MATERIAL AND METHODS: We performed a retrospective cross-sectional study. Based on the database of the General Surgery Division of the Prof Alejandro Posadas National Hospital, between July 2007and June 2013, charts of all patients with biliary lithiasis disease operated in this period were analyzed. We analyzed the clinical features, the various diagnostic techniques and the treatment carried out in each of them. RESULTS: Surgery due to biliary lithiasis was performed in 2,160 patients. Fourteen patients, 8 females and 6 males, had Mirizzi syndrome (0.65%). The mean age was 55.3 years old (range 34-70 years old). Four patients were scheduled for elective surgery and 10 were operated during emergency. Preoperative differential diagnosis was: extrahepatic cholestasis 10, cholecystitis 3 and biliary duct stenosis vs gallbladder cancer 1. The treatments performed were: 3 conventional cholecystectomies with intraoperative dynamic cholangiography, 2 conventional cholecystectomies plus choledochoplasty with Kehr tube, 2 laparoscopic cholecystectomies plus choledochoplasty with Kehr tube, 1 partial cholecystectomy with Pezzer catheterization, 3 choledochoduodenostomy, 1 choledochoplasty with gallbladder remnant, 1 hepaticojejunostomy and 1 treatment in two steps (percutaneous biliary drainage and cholecystectomy). Regarding complications, we observed 1 autolimited leak from the choledocoduodenostomy, 1 death in an ederly patient, and 1 hepatic abscess treated by a minimaly invasive approach. CONCLUSIONS: Mirizzi syndrome is a disease with low prevalence, which must be taken into account in biliary surgery, because the treatment varies according to the intraoperative findings and the experience of the surgical team.

[Primary sclerosing cholangitis presenting as recurrent cholangitis and right hepatic duct outpouching]. / Quiste de la vía biliar y colangitis de repetición como manifestaciones iniciales de colangitis esclerosante primaria.

Primary sclerosing cholangitis (PSC) may have an atypical mode of presentation with recurrent cholangitis and diverticulum-like outpouchings of the hepatic ducts; a high clinical suspicion is required to confirm the diagnosis instead of ascribing cirrhosis to a secondary cause as a result of the biliary stasis propitiated by the biliary cysts. We describe the case of a 63-year old woman with a one-year history of episodes of cholangitis and a persistent elevation of alkaline phosphatase. The endoscopic retrograde cholangiopancreatography and a magnetic resonance cholangiography showed strictures of the intrahepatic biliary tract compatible with PSC and a diverticulum-like outpouching of the right hepatic duct. A liver biopsy revealed cirrhosis. Initial management consisted of antibiotics, a sphincterotomy and the placement of a biliary plastic stent, however, the patient continued to have recurrent cholangitis and finally, the cyst was resected.

Biliary tract schwannoma: a rare cause of obstructive jaundice in a young patient.

Schwannoma is a tumor derived from Schwann cells which usually arises in the upper extremities, trunk, head and neck, retroperitoneum, mediastinum, pelvis, and peritoneum. However, it can arise in the gastrointestinal tract, including biliary tract. We present a 24-year-old male patient with obstructive jaundice, whose investigation with computed tomography abdomen showed focal wall thickening in the common hepatic duct, difficult to differentiate with hilar adenocarcinoma. He was diagnosed intraoperatively schwannoma of common bile duct and treated with local resection. The patient recovered well without signs of recurrence of the lesion after 12 mo. We also reviewed the common bile duct schwannoma related in the literature and evaluated the difficulty in pre and intraoperative differential diagnosis with adenocarcinoma hilar. Resection is the treatment of choice for such cases and the tumor did not recur in any of the resected cases.

Liver histological, portal flow and plasmatic nitric oxide alterations caused by biliary obstruction and drainage in rats.

PURPOSE: To evaluate liver alterations caused by biliary obstruction and drainage. METHODS: Thirty-nine male Wistar rats were randomly distributed in 4 groups: BO (n=18) bile duct ligation for 20 days, with a periodic evaluation of liver histological alterations, Doppler echography portal flow and measurements of NO and malondialdehyde (MDA); BO/DB (n=13) bile duct occlusion for 20 days followed by biliary drainage by choledochoduodenal anastomosis, 5 days follow-up, same BO group parameters evaluations; group CED (n=4) sham operation and portal flow evaluation trough 20 days; CHB (n=4) sham operation, with hepatic biopsy on 25th day and followed-up trough 25 days, by the same parameters of group BO, with exception of portal flow. Direct bilirubin (DB) and alkaline phosphatase (AP) were evaluated in the group BO, BO/DB and CHB. RESULTS: The bile duct ligation led to an increase of DB and AP, development of liver histological alterations, reduction of portal flow and increase of plasmatic NO and of MDA levels. The bile duct clearing resulted in a reduction of DB, AP, NO, MDA histological alterations and increase of portal flow. CONCLUSION: The biliary occlusion resulted in cholestasis and portal flow reduction, besides the increase of plasmatic NO and of hepatic MDA levels, and histological liver alterations, with a tendency of normalization after the bile duct clearing.

Liver histological, portal flow and plasmatic nitric oxide alterations caused by biliary obstruction and drainage in rats / Alterações histológicas, do fluxo portal e do óxido nítrico plasmático em ratos submetidos à obstrução/desobstrução biliar

PURPOSE: To evaluate liver alterations caused by biliary obstruction and drainage. METHODS: Thirty-nine male Wistar rats were randomly distributed in 4 groups: BO (n=18) bile duct ligation for 20 days, with a periodic evaluation of liver histological alterations, Doppler echography portal flow and measurements of NO and malondialdehyde (MDA); BO/DB (n=13) bile duct occlusion for 20 days followed by biliary drainage by choledochoduodenal anastomosis, 5 days follow-up, same BO group parameters evaluations; group CED (n=4) sham operation and portal flow evaluation trough 20 days; CHB (n=4) sham operation, with hepatic biopsy on 25th day and followed-up trough 25 days, by the same parameters of group BO, with exception of portal flow. Direct bilirubin (DB) and alkaline phosphatase (AP) were evaluated in the group BO, BO/DB and CHB. RESULTS: The bile duct ligation led to an increase of DB and AP, development of liver histological alterations, reduction of portal flow and increase of plasmatic NO and of MDA levels. The bile duct clearing resulted in a reduction of DB, AP, NO, MDA histological alterations and increase of portal flow. CONCLUSION: The biliary occlusion resulted in cholestasis and portal flow reduction, besides the increase of plasmatic NO and of hepatic MDA levels, and histological liver alterations, with a tendency of normalization after the bile duct clearing.

OBJETIVO: Avaliar alterações hepáticas resultantes do processo de obstrução/desobstrução biliar. MÉTODOS: Trinta e nove ratos Wistar foram distribuídos aleatoriamente em 4 grupos: OB (n=18), oclusão biliar, seguimento de 20 dias, avaliação das alterações histológicas hepáticas, do fluxo portal e dosagens de NO e de malondialdeído (MDA); grupo OB/DB (n=13) oclusão biliar por 20 dias seguida de desobstrução biliar por anastomose colédoco-duodenal, seguimento por 5 dias e avaliação dos mesmos parâmetros do grupo BO; grupo CED (n=4) operação simulada, avaliação do fluxo portal e seguimento por 25 dias; grupo CHB (n=4) operação simulada com biópsia hepática no 25º dia, e seguido por 25 dias com avaliação periódica dos mesmos parâmetros do grupo BO, com exceção da ecografia Doppler. Nos grupos BO, OB/DB e CHB foram avaliadas também a bilirrubina direta (BD) e a fosfatase alcalina (FA). RESULTADOS: A oclusão do colédoco causou aumento da BD e da FA, desenvolvimento de alterações hepáticas, redução do fluxo portal, com aumentos do NO plasmático e do MDA. A desobstrução biliar resultou na redução da BD, da FA, das alterações hepáticas, aumento do fluxo portal e redução do NO e do MDA. CONCLUSÃO: A obstrução biliar resultou em colestase, alterações histológicas hepáticas, diminuição do fluxo portal, aumentos do NO plasmático e MDA hepático, com tendência à normalização após a desobstrução biliar.

Plexiform neurofibroma: a rare cause of obstructive jaundice.

A case of obstructive jaundice secondary to a neurofibroma in the common hepatic duct is presented. The histological appearance was that of a plexiform neurofibroma. The clinicopathological features are discussed.

[Surgical treatment of Klatskin's tumor]. / Tratamiento quirúrgico del tumor de Klatskin.

Usually primary cancer originating in the hepatic duct confluence (Klatskin Tumor) is regarded as unresectable at diagnosis and therefore percutaneously or endoscopically placed stents have been advocated. Unfortunately with these palliative modalities the median survival is only 3-6 months. However with aggressive surgical resection of the tumor 17% of five year survival have been obtained. The present paper summarizes our experience from three patients with Klatskin tumor which underwent surgery. The Klatskin tumors were Type II (one case), Type IIIa (one case) and Type IIIb (one case). The patient with tumor Type II had wide tumor excision and extrahepatic bile duct resection at the liver hilum. The patients with tumor Type III had extrahepatic bile duct resection at the liver hilum with left and right hepatectomy respectively. Reconstruction was made with Roux-en-Y biliary enteric anastomosis, the mean post operative stay was 9 days. The mean post operative survival of two patients undergoing surgery with curative intent was 21 months, in contrast to 10 months for the patient with palliative surgery. One patient is alive 22 months with no evidence of disease. Our results supports an aggressive surgical approach in patients with Klatskin tumor which should include Hepatectomy to obtain free resection margins.

Adenomatosis del hepatocolédoco y ampulla / Adenomatosis of the hepatocholedocal duct ampulla

Se presentan 6 enfermos con obstrucción de la vía biliar extrahepática por adenomiosis. Esta patología se ubica en el grupo de los tumores epiteliais benignos, que obstruyen la vía biliar por la presencia intraparietal de cúmulos de conductos glandulares nodeados por un proceso inflamatorio crónico escleroso. Es una lesión de difícil diagnóstico clínico y operatorio donde la verdadera naturaleza la establece la anatomía patológica, que debe diferenciarlo del cáncer y páncreas aberrante

Adenomatosis del hepatocolédoco y ampulla / Adenomatosis of the hepatocholedocal duct ampulla

Se presentan 6 enfermos con obstrucción de la vía biliar extrahepática por adenomiosis. Esta patología se ubica en el grupo de los tumores epiteliais benignos, que obstruyen la vía biliar por la presencia intraparietal de cúmulos de conductos glandulares nodeados por un proceso inflamatorio crónico escleroso. Es una lesión de difícil diagnóstico clínico y operatorio donde la verdadera naturaleza la establece la anatomía patológica, que debe diferenciarlo del cáncer y páncreas aberrante (AU)

Histopathologic study of the liver in the early cholestatic phase of alpha-1-antitrypsin deficiency.

Liver biopsies obtained during the first six months of life were studied in 15 children who had prolonged neonatal cholestasis and alpha-1-antitrypsin deficiency (Pi ZZ). Intracellular PAS-positive globules were always observed, even during the first months of life. At this early stage of the disease, three morphologic patterns of hepatic alteration were distinguished. Group 1: six cases with cholestasis, hepatocellular damage, and slight portal fibrosis; Group 2: five cases with important portal fibrosis and bile duct proliferation; and Group 3: four cases in which ductular hypoplasia was the main feature. A correlation between histologic patterns and clinical course may be established: improvement of liver injury in Group 1, early cirrhosis in Group 2, and prolonged cholestasis in Group 3. The natural evolution of the disease appears to be different in each of the three groups.