A Rare Case of Simultaneous Pheochromocytoma Accompanied with an Ileocecal Neuroendocrine Tumor.

The detection rates of neuroendocrine tumors (NETs) are rising, which has led to a better understanding of their histopathologic characteristics. We present a case of adrenal pheochromocytoma with an incidental finding of a concurrent ileocecal NET detected on early post-adrenalectomy imaging. A 59-year-old male was noted to have microscopic hematuria on a routine workup. Further imaging identified a right adrenal lesion suggestive of pheochromocytoma. He was reporting intermittent headaches, and his urinary normetanephrine and norepinephrine levels were elevated. He underwent a right retroperitoneal adrenalectomy, and pathology confirmed a pheochromocytoma with no malignancy. Postoperatively, he developed a hematoma at the surgical bed that was identified on CT scan. An incidental lesion at the level of the ileocecal valve was noted on the CT scan as well. This was further investigated by colonoscopy and biopsy that confirmed a carcinoid tumor. He had no gastrointestinal symptoms, chromogranin A was normal, and 24-h urinary 5-hydroxyindolacetic acid levels were slightly elevated. He subsequently underwent a laparoscopic right hemicolectomy 3 months from his initial adrenalectomy. Final pathology confirmed a grade 2 NET with metastatic involvement of 4 regional lymph nodes. We report an extremely rare case of simultaneous pheochromocytoma accompanied with an ileocecal NET. Genetic testing for multiple endocrine neoplasia was undertaken and was found negative for 9 tested genes.

Solitary fibrous tumor of the greater omentum: case report and review of literature.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin occurring most often in the visceral pleura, however, it has been described in almost every anatomic location of the human body. While most SFTs have a benign behavior, they can potentially be locally aggressive and demonstrate a malignant behavior. CASE PRESENTATION: A 63 year-old male patient presented with lower abdominal pain and nausea and was noted on CT to have a large, heterogeneous lower abdominal mass with no evidence of metastatic disease. A surgical resection was performed and the mass appeared to be connected to the greater omentum with a vascular pedicle. It was not invading any intra-abdominal or pelvic organs. Pathology revealed an SFT of omental origin. The mitotic count was less than 4 per 10 high-power fields and all pathologic characteristics did not meet the criteria for a malignant SFT. CONCLUSIONS: We report an extremely rare case of SFT originating from the greater omentum. A multidisciplinary team approach was followed to plan the patient's management strategy.

Patterns of recurrence following selective intraoperative radiofrequency ablation as an adjunct to hepatic resection for colorectal liver metastases.

BACKGROUND AND OBJECTIVES: The purpose of this study was to analyze the patterns of recurrence following intraoperative radiofrequency ablation (RFA) combined with hepatic resection for patients with colorectal liver metastases (CLM). METHODS: Patients undergoing liver resection (with or without RFA) for CLM were examined. Rates and patterns of disease recurrence, as well as overall survival were assessed using Kaplan-Meier and Cox analyses. RESULTS: A total of 174 patients underwent liver resection for CLM (150 without and 24 with intraoperative RFA). RFA was used to treat 41 tumors (median 1.6 cm). The 3-year overall survival was 65.5% and 61.4% (adjusted HR 1.02, 95% CI 0.55-1.88). Median recurrence-free survival was 7.4 versus 12.7 months with RFA versus non-RFA, respectively (adjusted HR 1.51, 95% CI 0.94-4.42). On multivariate analysis, neither survival nor recurrence-free survival was significantly associated with RFA. In total, there were two RFA ablation zone local failures. An ablation site recurrence was the sole site in one patient (4.2%). CONCLUSION: RFA was used as an adjunct to resection in patients with greater disease burden. Despite this, RFA was not significantly associated with a higher risk of local failure and was not associated with worse survival, when compared with liver resection alone.

Effect of obesity and decompressive laparotomy on mortality in acute pancreatitis requiring intensive care unit admission.

BACKGROUND: Controversy still exists on the effect that obesity has on the morbidity and mortality in severe acute pancreatitis (SAP). The primary purpose of this study was to compare the mortality rate of obese versus nonobese patients admitted to the ICU for SAP. Secondary goals were to assess the potential risk factors for abdominal compartment syndrome (ACS) and to investigate the performance of validated scoring systems to predict ACS and in-hospital mortality. METHODS: A retrospective cohort of adults admitted to the ICU for SAP was stratified by their body mass index (BMI) as obese and nonobese. The rates of morbidity, mortality, and ACS were compared by univariate and multivariate regression analyses. Areas under the curve (AUC) were used to evaluate the discriminating performance of severity scores and other selected variables to predict mortality and the risk of ACS. RESULT: Forty-five patients satisfied the inclusion criteria and 24 (53 %) were obese with similar characteristics to nonobese patients. Among all the subjects, 11 (24 %) died and 16 (35 %) developed ACS. In-hospital mortality was significantly lower for obese patients (12.5 vs. 38 %; P = 0.046) even though they seemed to develop ACS more frequently (41 vs. 28 %; P = 0.533). At multivariable analysis, age was the most significant factor associated with in-hospital mortality (odds ratio (OR) = 1.273; 95 % confidence interval (CI) 1.052-1.541; P = 0.013) and APACHE II and Glasgow-Imrie for the development of ACS (OR = 1.143; 95 % CI 1.012-1.292; P = 0.032 and OR = 1.221; 95 % CI 1.000-1.493; P = 0.05) respectively. Good discrimination for in-hospital mortality was observed for patients' age (AUC = 0.846) and number of comorbidities (AUC = 0.801). ACS was not adequately predicted by any of the clinical severity scores (AUC = 0.548-0.661). CONCLUSIONS: Patients' age was the most significant factor associated with mortality in patients affected by SAP. Higher APACHE II and Glasgow-Imrie scores were associated with the development of ACS, but their discrimination performance was unsatisfactory.

Liver abscess caused by toothpick and treated by laparoscopic left hepatic resection: case report and literature review.

Hepatic abscesses caused by ingested foreign bodies have been reported in the medical literature but represent very uncommon events. Extra-luminal migration of sawing needles and pins is the most common cause of perforation of the gastrointestinal tract associated with liver infections. Other non-metallic sharp objects such as animal bones and toothpicks have been described but are less frequent. The authors present a case of a 45-year-old woman who suffered from sepsis and a liver abscess because of the migration of a toothpick that lodged in the left hepatic lobe. Review of the literature on the pathogenesis and clinical management of liver abscesses caused by ingested foreign is presented.

Benign inflammatory pseudotumour mimicking extrahepatic bile duct cholangiocarcinoma in an adult man presenting with painless obstructive jaundice.

Inflammatory pseudotumours (IPTs) of the biliary tract are extremely rare and heterogeneous by aetiology and clinical presentation. They might cause biliary obstruction and mimic cholangiocarcinomas and their final diagnosis is usually achieved only after surgical excision. The most characteristic feature of IPT is the presence of chronic inflammatory cell infiltrates with variable degree of proliferating fibrous tissue. IPTs have the potential for recurrence even after resection and if untreated they can grow causing a variety of symptoms due to compression of the surrounding structures and organs. Despite the significant improvement of modern imaging techniques, preoperative distinction between IPTs of the biliary system and malignancies is extremely difficult. Histological diagnosis poses a clinical challenge because sampling is often suboptimal. Although rare, IPTs should be in the differential diagnosis of patients who present with painless jaundice and no other clinical symptoms or signs characteristics of cholangiocarcinomas.

Quality of life and survival analysis of patients undergoing transarterial chemoembolization for primary hepatic malignancies: a prospective cohort study.

INTRODUCTION: Transarterial chemoembolization (TACE) is indicated for primary hepatic tumours when resection or local ablation are not feasible. Patients undergoing TACE have a better survival than best supportive therapy. However, there is paucity of prospective studies on the quality of life (QOL) after TACE for primary hepatic malignancies, especially in the Western world. PURPOSE: The primary aim of the present study was to determine if TACE impacts on the QOL of patients affected by primary hepatic tumours, and to assess treatment efficacy in a prospective cohort of patients treated at a tertiary Canadian university medical centre. METHODS: From September 2005 to December 2010, 48 candidates for TACE underwent at least one TACE session. Data on their QOL, tumour response, serum alpha fetoprotein (AFP) and survival were prospectively collected every 3-4 months. RESULTS: The overall QOL of patients undergoing TACE did not decline during the first 12 months after treatment. A decline was observed in the physical health domain after the third TACE that coincided with the increasing size of the largest tumour and a rise in the serum AFP levels. Psychological, social and environmental domains remained stable throughout the treatment period. Multivariate analysis revealed that tumour focality, AFP levels and model of end-stage liver disease (MELD) scores were associated with long-term survival (P= 0.001, P= 0.01, P= 0.02, respectively). The overall survival at 12, 36 and 48 months were 72%, 28% and 12%, respectively. CONCLUSION: TACE is an effective palliative intervention for unresectable and non-ablatable primary liver tumours without affecting the QOL of patients even when repeated interventions are performed.

Meta-analysis of phase III randomized trials of molecular targeted therapies for advanced pancreatic cancer.

OBJECTIVES: For patients with unresectable pancreatic cancer (PC), the efficacy and safety of molecular targeted agents (MTAs) in combination with gemcitabine are still unclear. Published randomized controlled trials (RCTs) have reported conflicting results. This study aimed to conduct a systematic review of the literature and to perform a meta-analysis if appropriate. METHODS: Seven electronic databases were searched using a standard technique to November 2011 without restriction on publication status or language. The primary aim was to assess overall survival (OS). Secondary aims were to assess progression-free survival (PFS), overall response rates (ORRs) and grade 3, 4 and 5 toxicities. A random-effects model was used for the meta-analysis. RESULTS: Seven Phase III RCTs were identified; 1981 patients were treated with MTAs and gemcitabine, and 1992 patients received gemcitabine with or without placebo. No statistically significant difference in OS was found between the two groups [hazard ratio (HR) = 0.93, 95% confidence interval (CI) 0.85-1.02; P = 0.13]. The addition of MTAs improved PFS (HR = 0.86, 95% CI 0.79-0.93; P = 0.000) and ORR (odds ratio 1.35, 95% CI 1.05-1.74; P = 0.01). However, these benefits were accompanied by significantly higher toxicity (P = 0.001). CONCLUSIONS: The findings of this study suggest that the palliation of PC with gemcitabine and MTAs does not provide a significant survival benefit and is associated with increased grade 3 and 4 toxicities.

Rifaximin vs. conventional oral therapy for hepatic encephalopathy: a meta-analysis.

AIM: To characterize the efficacy of rifaximin in the management of hepatic encephalopathy (HE) as several randomized controlled studies have shown contradictory results on its effectiveness in comparison to other oral agents. METHODS: We performed a systematic review and random effects meta-analysis of all eligible trials identified through electronic and manual searches. Twelve randomized controlled trials met the inclusion criteria with a total of 565 patients. RESULTS: The clinical effectiveness of rifaximin was equivalent to disaccharides or other oral antibiotics [odds ratio (OR) 0.96; 95% CI: 0.94-4.08] but with a better safety profile (OR 0.27; 95% CI: 0.12-0.59). At the completion of treatment protocols, patients receiving rifaximin showed lower serum ammonia levels [weighted mean difference (WMD) = -10.65; 95% CI: -23.4-2.1; P = 0.10], better mental status (WMD = -0.24; 95% CI: -0.57-0.08; P = 0.15) and less asterixis (WMD -0.1; 95% CI -0.26-0.07; P = 0.25) without reaching statistical significance. On the other hand, other psychometric outcomes such as electroencephalographic response and grades of portosystemic encephalopathy were superior in patients treated with rifaximin in comparison to the control group (WMD = 0.21, 95% CI: -0.33-0.09, P = 0.0004; and WMD = -2.33, 95% CI: -2.68-1.98, P = 0.00001, respectively). Subgroup and sensitivity analysis did not show any significant difference in the above findings. CONCLUSION: Rifaximin appears to be at least as effective as other conventional oral agents for the treatment of HE with a better safety profile.

Analysis of survival predictors in a prospective cohort of patients undergoing transarterial chemoembolization for hepatocellular carcinoma in a single Canadian centre.

BACKGROUND: Despite advances in the treatment of hepatocellular carcinoma (HCC), a great proportion of patients are eligible only for palliative therapy for reasons of advanced-stage disease or poor hepatic reserve. The use of transarterial chemoembolization (TACE) in the palliation of non-resectable HCC has shown a survival benefit in European and Asian populations. The aim of this study was to assess the efficacy of TACE by analysing overall 5-year survival, interval changes of tumour size and serum alpha-fetoprotein (AFP) levels in a prospective North American cohort. METHODS: From September 2005 to December 2010, 46 candidates for TACE were enrolled in the study. Collectively, they underwent 102 TACE treatments. Data on tumour response, serum AFP and survival were prospectively collected. RESULTS: In compensated cirrhotic patients, serial treatment with TACE had a stabilizing effect on tumour size and reduced serum AFP levels during the first 12 months. Overall survival rates at 1, 2 and 3 years were 69%, 58% and 20%, respectively. Younger individuals and patients with a lower body mass index, affected by early-stage HCC with involvement of a single lobe, had better survival in univariate analysis. After adjustment for risk factors, early tumour stage (T1 and T2 vs. T3 and T4) at diagnosis was the only statistically significant predictor for survival. CONCLUSIONS: In compensated cirrhotic patients, TACE is an effective palliative intervention and HCC stage at diagnosis seems to be the most important predictor of longterm outcomes.

Advances in diagnosis, treatment and palliation of pancreatic carcinoma: 1990-2010.

Several advances in genetics, diagnosis and palliation of pancreatic cancer (PC) have occurred in the last decades. A multidisciplinary approach to this disease is therefore recommended. PC is relatively common as it is the fourth leading cause of cancer related mortality. Most patients present with obstructive jaundice, epigastric or back pain, weight loss and anorexia. Despite improvements in diagnostic modalities, the majority of cases are still detected in advanced stages. The only curative treatment for PC remains surgical resection. No more than 20% of patients are candidates for surgery at the time of diagnosis and survival remains quite poor as adjuvant therapies are not very effective. A small percentage of patients with borderline non-resectable PC might benefit from neo-adjuvant chemoradiation therapy enabling them to undergo resection; however, randomized controlled studies are needed to prove the benefits of this strategy. Patients with unresectable PC benefit from palliative interventions such as biliary decompression and celiac plexus block. Further clinical trials to evaluate new chemo and radiation protocols as well as identification of genetic markers for PC are needed to improve the overall survival of patients affected by PC, as the current overall 5-year survival rate of patients affected by PC is still less than 5%. The aim of this article is to review the most recent high quality literature on this topic.

Differentiating the impact of anatomic and non-anatomic liver resection on early recurrence in patients with Hepatocellular Carcinoma.

BACKGROUND: For Hepatocellular Carcinoma (HCC) treated with hepatectomy, the extent of the resection margin remains controversial and data available on its effect on early tumor recurrence are very few and contradictory. The purpose of this study was to compare the impact of the type of resection (anatomic versus non-anatomic) on early intra-hepatic HCC recurrence in patients with solitary HCC and preserved liver function. METHODS: Among 53 patients with similar clinico-pathologic data who underwent curative liver resection for HCC between 2000 and 2006, 28 patients underwent anatomic resection of at least one liver segment and 25 patients underwent limited resection with a margin of at least 1 cm. RESULTS: After a close follow-up period of 24 months, no difference was detected in recurrence rates between the anatomic (35.7%) and the non-anatomic (40%) groups in either univariate (p = 0.74) and multivariate (p = 0.65) analysis. Factors contributing to early recurrence were tumor size (p = 0.012) and tumor stage including vascular invasion (p = 0.009). CONCLUSION: The choice of the type of resection for HCC should be based on the maintenance of adequate hepatic reserve. The type of resection (anatomic vs non-anatomic) was found not to be a risk factor for early tumor recurrence.

Neuroendocrine tumors of the gallbladder: an evaluation and reassessment of management strategy.

BACKGROUND: Gallbladder neuroendocrine tumors (GB-NETs) represent only 0.5% of all NETs, and little is known about their biological behavior. We sought to provide an overview of the current state of knowledge about GB-NETs and provide a recommendation for management. STUDY: A PubMed search was undertaken using the following criteria: primary gallbladder and carcinoid or NET. We also interrogated the SEER 9, 13 and 17 registries (1973 to 2005) and the Niigata registry (2003) to assess the epidemiology and clinicopathological characteristics. Finally, we compared the clinical presentation, management and prognosis of GB-NETs to that of gallbladder adenocarcinoma. RESULTS: GB-NETs probably derive from either a multipotent stem cell or neuroendocrine cells in intestinal or gastric metaplasia of the gallbladder epithelium, which occurs consequent upon cholelithiasis/chronic inflammation. Clinically and at surgery, GB-NETs are indistinguishable from gallbladder cancer (GBC) and "carcinoid syndrome" is evident in only ∼1%.The median survival was only 9.8 months among 278 cases of GB-NETs reported in SEER. The 5 year survival rate for tumors classified as carcinoids/neuroendocrine carcinoma or small cell cancer (SCC) was 36.9 and 0%, respectively. Soga divided GB-NETs into typical and variant carcinoids with 5 year survival rates of 60.4% and 21.3% respectively. CONCLUSIONS: GB-NETs have an aggressive behavior, and once diagnosed, extensive surgical management and careful NET follow up with CT scan is mandatory to facilitate early detection of recurrence. Since more aggressive surgical management for GBC has shown increased survival rates for these tumors, a similar strategy seems reasonable for GB-NETs. However, in high grade metastatic tumors, the primary management is mainly medical.