Synchronous Gall Bladder and Bile Duct Cancer: A Short Series of Seven Cases and a Brief Review of Literature.

BACKGROUND: Simultaneous presence of cancer in the gall bladder and in the biliary tree could be due to local spread, metastases, de novo multifocal origin, or as part of a field change. In the past, such an association has been described in patients with anomalous pancreatico-biliary ductal junction. AIMS: We studied seven consecutive patients with simultaneous gall bladder and bile duct malignancy with a view to identify the best way to treat them, and if possible to hypothesize the etiopathogenesis. METHODS: Over a period of 24 months, there were seven cases, with synchronous gall bladder and extra-hepatic bile duct cancer. RESULTS: None of our patients had anomalous pancreatico-biliary ductal junction. Three patients were found to have inoperable disease, three other underwent curative resection, and one patient had a complete response to chemotherapy. Herein, we describe these patients and our lessons learnt from these patients with synchronous bile duct and gall bladder cancer. Of the seven patients, we were able to complete a curative resection in three patients, and the three patients were found to have inoperable disease. One patient had an excellent response to chemotherapy. CONCLUSION: Thus aggressive therapy in such patients with gall bladder cancer may be warranted in select cases. Also, the gall bladder specimens in patients undergoing surgery for cholangiocarcinoma should be analyzed in detail to identify foci of dysplasia or change in the epithelium. The pathogenesis may be due to a common field change in the biliary epithelium.

Rectal adenocarcinoma coexisting with gastro-intestinal stromal tumor: a case report and literature review.

The finding of a gastrointestinal stromal tumor along with other epithelial cancers has been previously reported. Most commonly occurring in the GI tract, a second malignancy has been reported in nearly 13-20% patients with GIST. An elderly woman with a moderately differentiated adenocarcinoma of the rectum underwent low anterior resection. Histology revealed a low-grade GIST along with adenocarcinoma of the rectum, with no lymph node involvement or metastatic disease. This seems to be the first case of a simultaneous occurrence of a GIST along with a rectal adenocarcinoma. The high incidence of a second malignancy in patients with GIST points toward an increased susceptibility to cancer. Is it necessary to treat such patients as generalized cancer syndromes with intensive surveillance and cancer screening?

A guide to the management of tuberculosis in patients with chronic liver disease.

Tuberculosis remains one of the 'Captains of the Men of Death' even today, particularly in the developing world. Its frequency is increased 14-fold in patients with chronic liver diseases (CLD) and liver cirrhosis, more so in those with decompensated disease, probably due to the cirrhosis-associated immune dysfunction syndrome, and case-fatality rates are high. The diagnosis of tuberculosis, particularly the interpretation of the Mantoux test, is also fraught with difficulties in CLD, especially after previous BCG vaccination. However, the greatest challenge in the patient with CLD or liver cirrhosis and tuberculosis is managing their therapy since the best first-line anti-tuberculosis drugs are hepatotoxic and baseline liver function is often deranged. Frequency of hepatotoxicity is increased in those with liver cirrhosis, chronic hepatitis B and chronic hepatitis C, possibly related to increased viral loads and may be decreased following antiviral therapy. If hepatotoxicity develops in those with liver cirrhosis, particularly decompensated cirrhosis, the risk of severe liver failure is markedly increased. Currently, there are no established guidelines for anti-tuberculosis therapy (ATT) in CLD and liver cirrhosis although the need for such guidelines is self-evident. It is proposed that ATT should include no more than 2 hepatotoxic drugs (RIF and INH) in patients with CLD or liver cirrhosis and stable liver function [Child-Turcotte-Pugh (CTP) ≤7], only a single hepatotoxic drug (RIF or INH) in those with advanced liver dysfunction (CTP 8-10) and no hepatotoxic drugs with very advanced liver dysfunction (CTP ≥11). A standard protocol should be followed for monitoring ATT-related hepatotoxicity and for stop rules and reintroduction rules in all these patients, on the lines proposed here. It is hoped that these proposals will introduce uniformity and result in streamlining the management of these difficult patients.

Selective internal radiation therapy for gastrointestinal neuroendocrine tumour liver metastases: a new and effective modality for treatment.

Background. Nonresectable neuroendocrine tumour (NET) liver metastases respond poorly to most widely available and used therapies. Selective Internal Radiation Therapy (SIRT) is becoming recognized as a new modality for selectively treating non-resectable liver tumours. This paper presents an experience of 14 patients with non-resectable NET liver metastases treated with SIRT. Methods. Between September 1997 and October 2009 14 patients with extensive NET liver metastases were treated with 2.0 to 3.0 GBq of (90)Yttrium microspheres. Repeat SIRT was undertaken in three patients after 16, 27, and 48 months, respectively. Responses were assessed clinically, biochemically, and with serial CT scans. Survival was measured from initial SIRT. Results. Some response was seen in all 14 patients. Carcinoid syndrome improved or resolved in 10/10 instances. 24-hour urinary 5-HIAA or serum chromogranin A levels fell dramatically in 5/7 patients following SIRT. Serial CT scans revealed partial response or stable disease in all 14 patients. Repeat treatment in three patients experiencing progression was associated with a further response. Median survival after SIRT is 25 months with 6 patients being alive (and 3 patients still asymptomatic), at 19, 22, 23, 23, 58, and 60 months. Conclusions. SIRT is an effective and well-tolerated treatment for non-resectable NET liver metastases capable of both alleviating the carcinoid syndrome and achieving significant tumour regression. Repeat treatment is an option and liver resection after downstaging may also become possible.

Terlipressin in hepatorenal syndrome: Evidence for present indications.

Hepatorenal syndrome (HRS) is the most frequent life threatening complication of advanced liver failure and cirrhosis. HRS results from a functional renal dysfunction due to circulatory disturbances in patients with advanced liver disease and portal hypertension. Reduction in the effective circulating blood volume and hence hypoperfusion of the kidney is the basic underlying common pathogenetic mechanism for the development of hepatorenal syndrome. The prognosis for HRS remains very poor with types 1 and 2-both having an expected survival time of 2 weeks and 6 months, respectively. Although the available data are derived from studies including a limited number of patients mainly affected by type 1 HRS, vasoconstrictor drugs, in particular the vasopressin analog Terlipressin, seem to be the most effective approach for the management of HRS. Associated with albumin infusion, these drugs have been shown to lead to reduced mortality and improved renal function in HRS. Terlipressin administration significantly increases mean arterial pressure and systemic vascular resistance; while the heart rate, cardiac output, HVPG and portal venous blood flow decrease significantly. This decrease correlates well with the decrease in plasma renin activity. Thus the vasoconstrictor effect of Terlipressin reverses the basic pathology of HRS by reducing the plasma renin activity. The improvement in hemodynamics with Terlipressin is associated with an increase in glomerular filtration rate and deactivation of the vasoconstrictor and sodium-conserving hormones with reduced activity of the RAAS resulting in increased natriuresis. Terlipressin thus reverses HRS and is useful in bridging the patient to liver transplantation and may hence indirectly improve survival. Patients with HRS who show an improvement in renal function with Terlipressin and albumin seem to have an excellent post-transplantation outcome similar to that of patients without HRS. Thus, the use of Terlipressin has been shown to be safe, with minimal side effects that usually disappear after dose reduction, and results in an improved outcome in patients with HRS.

Noncirrhotic portal hypertension.

Portal hypertension is characterized by an increase in portal pressure (> 10 mmHg) and could be a result of cirrhosis of the liver or of noncirrhotic diseases. When portal hypertension occurs in the absence of liver cirrhosis, noncirrhotic portal hypertension (NCPH) must be considered. The prognosis of this disease is much better than that of cirrhosis. Noncirrhotic diseases are the common cause of portal hypertension in developing countries, especially in Asia. NCPH is a heterogeneous group of diseases that is due to intrahepatic or extrahepatic etiologies. In general, the lesions in NCPH are vascular in nature and can be classified based on the site of resistance to blood flow. In most cases, these disorders can be explained by endothelial cell lesions, intimal thickening, thrombotic obliterations, or scarring of the intrahepatic portal or hepatic venous circulation. Many different conditions can determine NCPH through the association of these various lesions in various degrees. Many clinical manifestations of NCPH result from the secondary effects of portal hypertension. Patients with NCPH present with upper gastrointestinal bleeding, splenomegaly, ascites after gastrointestinal bleeding, features of hypersplenism, growth retardation, and jaundice due to portal hypertensive biliopathy. Other sequelae include hyperdynamic circulation, pulmonary complications, and other effects of portosystemic collateral circulation like portosystemic encephalopathy. At present, pharmacologic and endoscopic treatments are the treatments of choice for portal hypertension. The therapy of all disorders causing NCPH involves the reduction of portal pressure by pharmacotherapy or portosystemic shunting, apart from prevention and treatment of complications of portal hypertension.

Spontaneous bacterial peritonitis from Salmonella: an unusual bacterium with unusual presentation.

Spontaneous bacterial peritonitis (SBP) is a common cause of morbidity and mortality in patients with advanced cirrhosis and portal hypertension. While gram-negative rods and Enterococcus species are the common offending organisms, Salmonella has also been recognized as a rare and atypical offending organism. Atypical features of Salmonella SBP include both its occurrence in cirrhotic patients with immunosuppressive state and its lack of typical neutroascitic response. Diagnosis is often delayed as it requires confirmation from ascitic fluid culture. We report a case of Salmonella SBP occurring in a patient with decompensated cryptogenic cirrhosis with concurrent low-grade non-Hodgkin lymphoma and prior treatment with rituximab. Physicians should be aware of the atypical presentation, especially in cirrhotic patients who are immunosuppressed.