Ewing's sarcoma of the duodenum: a rare clinical condition managed with surgical resection.

A man in his 40s with no medical history presented with right-sided abdominal and chest pain. A CT scan of the abdomen demonstrated a 7.7 cm heterogeneous mass arising from the second part of the duodenum. Oesophagogastroduodenoscopy confirmed a malignant-appearing duodenal lesion, with biopsy showing features consistent with small cell carcinoma. The patient underwent three cycles of neoadjuvant chemotherapy, followed by elective Kausch-Whipple pancreaticoduodenectomy. A combination of immunohistochemistry and molecular studies confirmed the diagnosis of a rare Ewing's sarcoma tumour originating from the duodenum with invasion into the duodenal lumen. The patient recovered well from surgery and remains disease-free 18 months following resection.

Variation in survival after surgery for peri-ampullary cancer in a regional cancer network.

BACKGROUND: Centralisation of specialist surgical services requires that patients are referred to a regional centre for surgery. This process may disadvantage patients who live far from the regional centre or are referred from other hospitals by making referral less likely and by delaying treatment, thereby allowing tumour progression. The aim of this study is to explore the outcome of surgery for peri-ampullary cancer (PC) with respect to referring hospital and travel distance for treatment within a network served by five hospitals. METHODS: Review of a unit database was undertaken of patients undergoing surgery for PC between January 2006 and May 2014. RESULTS: 394 patients were studied. Although both the median travel distance for patients from the five hospitals (10.8, 86, 78.8, 54.7 and 89.2 km) (p < 0.05), and the annual operation rate for PC (2.99, 3.29, 2.13, 3.32 and 3.07 per 100,000) (p = 0.044) were significantly different, no correlation was noted between patient travel distance and population operation rate at each hospital. No difference was noted between patients from each hospital in terms of resection completion rate or pathological stage of the resected tumours. The median survival after diagnosis for patients referred from different hospitals ranged from 1.2 to 1.7 years and regression analysis revealed that increased travel distance to the regional centre was associated with a small survival advantage. CONCLUSION: Although variation in the provision and outcome of surgery for PC between regional hospitals is noted, this is not adversely affected by geographical isolation from the regional centre. TRIAL REGISTRATION: This study is part of post-graduate research degree project. The study is registered with ClinicalTrials.gov (unique identifier NCT02296736 ) November 18, 2014.

Rebound growth of hepatic colorectal metastases after neo-adjuvant chemotherapy: effect on survival after resection.

BACKGROUND: A period of recovery is commonly allowed between completion of chemotherapy for colorectal liver metastases (CRLM) and resection, during which tumour progression may occur. The study-aim is to assess the growth of CRLM in this interval and association with outcome. METHOD: Data on 146 patients were analysed. Change in tumour size was assessed by comparing size determined by imaging performed on completion of chemotherapy with that determined by examination of the resected specimen, categorised by RECIST criteria. RESULTS: In the interval before surgery sixteen patients (11%) fulfilled criteria for partial response (PR), 48 (33%) had stable disease (SD) and 82 (56%) had progressive disease (PD). Among patients with PD following chemotherapy the median disease-free survival of patients who initially responded (26 months) was longer than in those who initially had stable disease (7 months) (P = 0.002). No association was noted between rate of tumour growth after completion of chemotherapy and disease-free survival. CONCLUSION: Change in tumour size after completion of chemotherapy is variable and can be rapid, especially in patients who initially respond to treatment. However, disease-free survival is determined by tumour behaviour during treatment and not by change in size after completion of chemotherapy.

Assessment of the effect of interval from presentation to surgery on outcome in patients with peri-ampullary malignancy.

BACKGROUND: Delay between diagnosis of peri-ampullary cancer (PC) and surgery may allow tumour progression and affect outcome. The aim of this study was to explore associations of interval to surgery (IS) with pathological outcomes and survival in patients with PC. METHOD: A database review of all patients undergoing surgery between 2006 and 2014 was undertaken. IS was measured from diagnosis by imaging. Potential association between IS and survival was measured using Cox regression analysis, and between IS and pathological outcome with multivariate logistic analysis. RESULTS: 388 patients underwent surgery. The median IS was 49 days (1-551 days), and was not associated with any of the evaluated outcomes in patients with pancreatic (149) or distal bile duct (46) cancer. For patients with ampullary cancer (71) longer IS was associated with improved survival, with median survival of 27.5 months for patients waiting ≤ median IS (35) and 38.3 months for patients waiting > median IS (36) for surgery (p = 0.041). A higher rate of margin positivity (31.4%) was also noted among patients who waited less than the median IS compared to those waiting longer than this interval (11.4%) (p = 0.032). CONCLUSION: For patients with ampullary cancer there is a paradoxical improvement in outcome among those with a longer IS, which may be explained by progression to inoperability of more aggressive lesions.

Clinical assessment before hepatectomy identifies high-risk patients.

BACKGROUND: Liver resection is associated with significant morbidity, and assessment of risk is an important part of preoperative consultations. Objective methods exist to assess operative risk, including cardiopulmonary exercise testing (CPX). Subjective assessment is also made in clinic, and patients perceived to be high-risk are referred for CPX at our institution. This article addresses clinicians' ability to identify patients with a higher risk of surgical complications after hepatectomy, using selection for CPX as a surrogate marker for increased operative risk. MATERIALS AND METHODS: Prospectively collected data on patients undergoing hepatectomy between February 2008 and November 2013 were retrieved and the cohort divided according to CPX referral. Complications were classified using the Clavien-Dindo system. RESULTS: CPX testing was carried out before 101 of 405 liver resections during the study period. The median age was 72 and 64 in CPX and non-CPX groups, respectively (P < 0.001). The resection size was similar between the groups. No difference was noted for grade III complications between CPX and non-CPX tested-groups; however, 19 (18.8%) and 28 (9.2%) patients suffered grade IV-V complications, respectively (P = 0.009). There was no difference in long-term survival between groups (P = 0.63). CONCLUSIONS: This study attempts to assess clinicians' ability to identify patients at greater risk of complications after hepatectomy. The confirmation that patients identified in this way are at greater risk of grade IV-V complications demonstrates the value of preoperative counseling. High-risk patients do not have worse long-term outcomes suggesting survival is determined by other factors, particularly disease recurrence.

Socioeconomic status influences the likelihood but not the outcome of liver resection for colorectal liver metastasis.

BACKGROUND: The aim of this study was to compare the socioeconomic profile of patients undergoing liver resection for colorectal liver metastasis (CLM) in a regional hepatopancreatobiliary unit with that of the local population. A further aim was to determine if degree of deprivation is associated with tumour recurrence after resection. METHODS: A retrospective analysis of patients undergoing liver resection for CLM was performed. Geodemographic segmentation was used to divide the population into five categories of socioeconomic status (SES). RESULTS: During a 7-year period, 303 patients underwent resection for CLM. The proportion of these patients in the two least deprived categories of SES was greater than that of the local population (50.2% versus 40.2%) and the proportion in the two most deprived categories was lower (18.3% versus 30.1%) (P < 0.001). There was no difference in recurrence rate (P = 0.867) or disease-free survival among categories of SES (P = 0.913). Multivariate analysis demonstrated no association between SES and tumour recurrence (P = 0.700). CONCLUSIONS: Liver resection for CLM is performed more commonly among the least socioeconomically deprived population than among the most deprived. However, degree of deprivation was not associated with tumour recurrence after resection.

Extended pathology reporting of resection specimens of colorectal liver metastases: the significance of a tumour pseudocapsule.

INTRODUCTION: The aim of this study was to analyse the influence of factors reported in the minimum histopathology dataset for colorectal liver metastases (CRLM) and other pre-operative factors compared with additional data relating to the presence of tumour pseudocapsules and necrosis on recurrence 1 year after a resection. METHODS: For a period of 14 months, extended histological reporting of CRLM specimens was performed, including the presence of pseudocapsules and necrosis in each tumour. The details of recurrence were obtained from surveillance imaging. RESULTS: In 66 patients there were 27 recurrences within 1 year. The rates were lower for patients with tumour pseudocapsules (8/27) than for patients without (19/36) (P = 0.030). Pseudocapsules were associated with a younger age (P = 0.005), nodal stage of the primary colorectal tumour (P = 0.025) and metachronous tumours (P = 0.004). In patients with synchronous disease and pseudocapsules, the recurrence rate was 2/12 compared with 13/23 patients without pseudocapsules (P = 0.026). DISCUSSION: These findings demonstrate that histological examination of resection specimens can provide significant additional prognostic information for patients after resection of CRLM, compared with clinical and radiological data. The present finding that the absence of a pseudocapsule in patients with synchronous CRLM is associated with a dramatically worse outcome may help direct patient-specific adjuvant treatment and care.

Serum arterial lactate concentration predicts mortality and organ dysfunction following liver resection.

BACKGROUND: The aim of this study was to determine if the post-operative serum arterial lactate concentration is associated with mortality, length of hospital stay or complications following hepatic resection. METHODS: Serum lactate concentration was recorded at the end of liver resection in a consecutive series of 488 patients over a seven-year period. Liver function, coagulation and electrolyte tests were performed post-operatively. Renal dysfunction was defined as a creatinine rise of >1.5x the pre-operative value. RESULTS: The median lactate was 2.8 mmol/L (0.6 to 16 mmol/L) and was elevated (≥2 mmol/L) in 72% of patients. The lactate concentration was associated with peak post-operative bilirubin, prothrombin time, renal dysfunction, length of hospital stay and 90-day mortality (P < 0.001). The 90-day mortality in patients with a post-operative lactate ≥6 mmol/L was 28% compared to 0.7% in those with lactate ≤2 mmol/L. Pre-operative diabetes, number of segments resected, the surgeon's assessment of liver parenchyma, blood loss and transfusion were independently associated with lactate concentration. CONCLUSIONS: Initial post-operative lactate concentration is a useful predictor of outcome following hepatic resection. Patients with normal post-operative lactate are unlikely to suffer significant hepatic or renal dysfunction and may not require intensive monitoring or critical care.

The effect of body position on compartmental intra-abdominal pressure following liver transplantation.

BACKGROUND: Current assumptions rely on intra-abdominal pressure (IAP) being uniform across the abdominal cavity. The abdominal contents are, however, a heterogeneous mix of solid, liquid and gas, and pressure transmission may not be uniform. The current study examines the upper and lower IAP following liver transplantation. METHODS: IAP was measured directly via intra-peritoneal catheters placed at the liver and outside the bladder. Compartmental pressure data were recorded at 10-min intervals for up to 72 h following surgery, and the effect of intermittent posture change on compartmental pressures was also studied. Pelvic intra-peritoneal pressure was compared to intra-bladder pressure measured via a FoleyManometer. RESULTS: A significant variation in upper and lower IAP of 18% was observed with a range of differences of 0 to 16 mmHg. A sustained difference in inter-compartmental pressure of 4 mmHg or more was present for 23% of the study time. Head-up positioning at 30° provided a protective effect on upper intra-abdominal pressure, resulting in a significant reduction in all patients. There was excellent agreement between intra-bladder and pelvic pressure. CONCLUSIONS: A clinically significant variation in inter-compartmental pressure exists following liver transplantation, which can be manipulated by changes to body position. The existence of regional pressure differences suggests that IAP monitoring at the bladder alone may under-diagnose intra-abdominal hypertension and abdominal compartment syndrome in these patients. The upper and lower abdomen may need to be considered as separate entities in certain conditions.

A case of widespread aortic thrombosis secondary to acute severe pancreatitis.

We offer this case for publication as we believe that this is the first report of widespread aortic thrombosis secondary to acute severe pancreatitis.

An uncommon cause of life-threatening gastrointestinal bleeding: 2 synchronous Dieulafoy lesions.

Dieulafoy lesions are a rather uncommon cause of gastrointestinal bleeding that can be torrential and life-threatening. Extragastric location and pediatric cases are very rare. We report the first case of synchronous Dieulafoy lesions in the stomach and jejunum. This case is discussed in the light of the reported literature on this condition.

A prospective study investigating the cost effectiveness of intraoperative blood salvage during liver transplantation.

BACKGROUND: Adult orthotopic liver transplantation is associated with significant use of allogenic blood products, which places considerable demands on finite resources. This could be reduced by autologous red cell salvage use, and we evaluated its cost effectiveness in this prospective study. METHODS: Intraoperative autotransfusion was used in 660 adult liver transplant patients between January 1997 and July 2002. These included 134 with acute liver failure, 62 retransplants, 90 alcohol-related, 183 viral, 98 cholestatic chronic liver diseases, and 93 with other etiologies. RESULTS: The total volume of red blood cells transfused was 3641+/-315 ml, 2805+/-234 ml, 2603+/-443 ml, and 2785+/-337 ml for alcohol-related, viral, cholestatic, and others, respectively. Low preoperative hemoglobin was significantly associated with higher intraoperative transfusion requirements. Blood volumes transfused at retransplantation were significantly higher (7077+/-1110 ml vs. 2864+/-138 ml; P<0.001) than for acute liver failure and chronic liver disease. Autologous blood volumes transfused were similar in all diagnostic groups, but were significantly greater in retransplantation (2754+/-541 ml vs. 1524+/-77 ml; P<0.01). Venovenous bypass was significantly associated with higher transfusion requirements. Total savings per case were similar for all diagnostic groups but were greater in cases of retransplantation (864+/-222 pounds (1235+/-317 US dollars) vs. 238+/-24 pounds (340+/-34 US dollars; P<0.001). With the use of autologous transfusion over the study period, a cost saving of 131,901 pounds (188,618 US dollars) was achieved. CONCLUSIONS: Intraoperative red blood cell salvage and autologous transfusion is cost effective in adult liver transplantation. Currently, where optimum resource utilization and fiscal constraint are paramount in healthcare delivery, autologous transfusion is an important adjunct in liver transplantation.

Long-term outcome of liver retransplantation in children.

BACKGROUND: Retransplantation of the liver is the only means of prolonging survival in children whose initial graft has failed. Patient and graft survival rates after retransplantation in most series have been inferior to rates after first transplantation. PATIENTS AND METHODS: Of 450 pediatric liver transplantations performed between January 1990 and March 2001, 50 were first retransplantations, 9 were second retransplantations, and 1 was a third retransplantation. The overall retransplantation rate was 13.3% (median age at retransplantation 4 years and median weight 15 kg). The median post-retransplantation follow-up was 73 (range, 6-139) months. RESULTS: Kaplan-Meier patient survival rates for the group (n=50) were 71.7%, 64.7%, and 64.7% at 1, 3, and 5 years, respectively. Graft survival rates were 65.6%, 56.7%, and 56.7% at 1, 3, and 5 years, respectively. This is significantly worse than rates for children undergoing first liver transplantation. There were 17 deaths, of which 9 occurred in the first month. Biliary complications occurred in 5 (10%) patients and vascular complications in 6 (12%). Improved patient and graft survival rates were observed in the later phase of the program, although the difference was not significant. Higher preoperative serum creatinine (P=0.001) and serum bilirubin (P=0.02) levels were associated with a higher postoperative mortality. CONCLUSION: Results of retransplantation in children remain inferior to those after first transplantation. There is a trend toward improving results. Liver retransplantation makes an important contribution to overall survival in children.

Results of split liver transplantation in children.

OBJECTIVE: To analyze the outcome of 80 consecutive pediatric split liver transplants performed at the authors' center between 1994 and 2000. SUMMARY BACKGROUND DATA: Split liver transplantation has become an accepted method of increasing the number of available grafts for pediatric liver transplant recipients. METHODS: The age of the patients at the time of transplantation ranged from 5 days to 16 years (median 3 years). Sixteen transplants were performed for acute liver failure and 64 for chronic liver failure. The ex situ splitting technique was used for all but four grafts. Fourteen livers were split for two pediatric recipients. Posttransplant follow-up ranged from 6 to 84 months (median 42 months). RESULTS: Overall patient survival at 6 months follow-up was 96.2%. Graft survival at six months was 93.7%. The Kaplan-Meier patient survival rates at 1 and 3 years were 93.5% and 88.1%, and the graft survival rates were 89.7% and 86.1%, respectively. Four patients required retransplantation. In the acute group (n = 16), the patient survival rates were 93.7% at 1 year and 76.4% at 3 years; there were three deaths due to posttransplant lymphoproliferative disease (PTLD), sepsis, and chronic rejection. In the chronic group (n = 64), the 1- and 3-year patient survival rates were 93.6% and 90.9%, respectively. There were six deaths in this group. Four patients died in the first year after the transplant due to intracranial bleeding, cerebral tumor recurrence, PTLD, and chronic rejection. There were two deaths at 3 years, one due to progressive renal failure secondary to cyclosporin toxicity and the other due to sepsis, portal hypertension, and recurrent bleeding. Vascular complications occurred in six (7.5%) patients and biliary complications in seven (8.7%). CONCLUSIONS: These results, which represent the experience of a single institution over the last 6 years, indicate that ex situ split liver transplantation can be performed in children with good overall outcome and acceptable morbidity.