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.

Serious complications of pancreatoduodenectomy correlate with lower rates of adjuvant chemotherapy: Would high-risk patients benefit from neoadjuvant therapy?

INTRODUCTION: Patients who suffer a serious complication of pancreatoduodenectomy (PD) may have their adjuvant chemotherapy (AC) delayed or omitted as a result. We aimed to investigate whether PD complications affected AC rates. MATERIALS AND METHODS: A retrospective analysis of all PD patients with histologically-confirmed pancreatic ductal adenocarcinoma (2006-2015) was performed; 90-day mortality patients were excluded. Patients who commenced AC were compared to those who did not (morbidity rates and survival) and patients who developed selected postoperative complications were compared to those who did not (AC rates and survival). RESULTS: 157 patients were included and 90-day mortality was 3.8%. Of the remaining patients, 102 (68.5%) received AC (AC data unavailable for two patients). Survival was longer in the AC group (p = 0.004). AC patients had less frequently experienced a postoperative chest infection (8.82% vs 34.0%, p = 0.0003) or a postoperative complication which was Clavien-Dindo (CD) grade ≥ II (29.4% vs 57.4%, p = 0.0019) or ≥ III (6.86% vs 21.3%, p = 0.023). Patients who experienced a postoperative chest infection (36.0% vs 75.0%, p = 0.0003) or a postoperative complication which was CD grade ≥ II (48.9% vs 73.1%, p = 0.0099) or ≥ III (29.4% vs 70.3%, p = 0.0018) less frequently commenced AC. CONCLUSION: Patients who received AC had less frequently experienced a serious postoperative complication. Efforts should be made to preoperatively identify those who are high-risk for a serious complication as this cohort may benefit from neoadjuvant therapy.

Impact of SARS-CoV-2 pandemic on pancreatic cancer services and treatment pathways: United Kingdom experience.

INTRODUCTION: The SARS-CoV-2 pandemic presented healthcare providers with an extreme challenge to provide cancer services. The impact upon the diagnostic and treatment capacity to treat pancreatic cancer is unclear. This study aimed to identify national variation in treatment pathways during the pandemic. METHODS: A survey was distributed to all United Kingdom pancreatic specialist centres, to assess diagnostic, therapeutic and interventional services availability, and alterations in treatment pathways. A repeating methodology enabled assessment over time as the pandemic evolved. RESULTS: Responses were received from all 29 centres. Over the first six weeks of the pandemic, less than a quarter of centres had normal availability of diagnostic pathways and a fifth of centres had no capacity whatsoever to undertake surgery. As the pandemic progressed services have gradually improved though most centres remain constrained to some degree. One third of centres changed their standard resectable pathway from surgery-first to neoadjuvant chemotherapy. Elderly patients, and those with COPD were less likely to be offered treatment during the pandemic. CONCLUSION: The COVID-19 pandemic has affected the capacity of the NHS to provide diagnostic and staging investigations for pancreatic cancer. The impact of revised treatment pathways has yet to be realised.

Chyle leak following pancreatico-duodenectomy: a tertiary hepatopancreaticobiliary unit's experience and a proposed management algorithm.

BACKGROUND: Chyle leak (CL) is an uncommon complication of pancreatico-duodenectomy (PD). Its incidence, risk factors, and effect on prognosis are not well described and optimum management remains debated. This study aims to calculate incidence of CL following PD and identify risk factors. Following a literature review, we have proposed a management algorithm. METHODS: This is a retrospective review of all patients who underwent PD between January 2006 and April 2020 at a tertiary hepatopancreaticobiliary unit in the UK. The following data were obtained: age, gender, American Society of Anesthesiologists grade, body mass index, co-morbidities, duration of surgery, tumour histology, length of stay and mortality. RESULTS: A total of 560 patients were included. Seventeen developed CL (3.04%). Median age was 64 years (range 50-81). Sixteen (94.1%) patients still had their surgical drain in at the time of CL diagnosis. One (5.9%) did not and had free intra-abdominal fluid on computed tomography; a diagnosis was made after an ultrasound-guided drain had been inserted. CL patients were more likely to have higher body mass index (mean 30.5 kg/m2 (range 17-43) versus 26.7 kg/m2 (22-38)) (P = 0.02) and longer duration of operation (mean 6.2 h (range 4.3-9.0) versus 5.6 (3.0-11.0)) (P = 0.03). All cases of CL resolved without operative intervention. CL did not affect length of stay (median 10 days (range 4-41) versus 11 (4-34)). CONCLUSIONS: In our series, 3.04% of patients who underwent PD developed CL. No patients required a return to theatre, and none had CL recurrence.

Comparison of lipase and amylase for diagnosing post-operative pancreatic fistulae.

BACKGROUND: To ascertain if post-operative drain fluid lipase is superior to amylase in routinely detecting clinically significant post-operative pancreatic fistulae (POPF). METHOD: Between January 2015 and March 2016 data were collected on all patients undergoing pancreatic surgery at a regional referral centre. Routine drain fluid analysis was performed on post-operative patients as part of a locally defined enhanced recovery protocol. POPF was diagnosed in accordance with the recently updated International Study Group of Pancreatic Surgery guidance. RESULTS: During the study period, there were 68 pancreatic resections. The median age was 69.1 years. A total of 11 (15.9%) patients developed clinically significant POPF (nine type B and two type C). The median drain amylase result in patients with type B or C leak was 532 IU/L (interquartile range (IQR) 264-833). This was significantly higher than those without a clinical fistula (median 38, IQR 15-376, P = 0.012). The median drain lipase result was 1504 IU/L (IQR 746-2236). This was significantly higher than those without a clinical fistula (median 57, IQR 13-1277, P = 0.012). Fluid amylase had a sensitivity of 81.8% and specificity of 69.2%; fluid lipase had a sensitivity of 91% and specificity of 64.9%. CONCLUSION: Our experience suggests drain fluid amylase or lipase results are not sufficiently sensitive or specific to reassure clinicians and rule out clinically significant POPF. However, if biochemical tests are used to aid decision-making, then lipase is a more sensitive biochemical marker than amylase for the routine detection of clinically significant POPF.

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.

Differentiating Simple Hepatic Cysts from Mucinous Cystic Neoplasms: Radiological Features, Cyst Fluid Tumour Marker Analysis and Multidisciplinary Team Outcomes.

BACKGROUND: Differentiating hepatic mucinous cystic neoplasms (MCNs) from simple hepatic cysts (SCs) preoperatively is a challenging task. Our aim was to determine whether radiological features on ultrasound scan (USS), CT or MRI, cyst fluid tumour markers, or multidisciplinary team (MDT) outcomes could differentiate MCN from SC. METHODS: A retrospective review of radiological features, cyst fluid tumour marker levels and MDT outcomes in 52 patients was performed. RESULTS: There were 13 patients with MCN, 38 with SC and one ciliated foregut cyst. MCNs were more often solitary (p = 0.006). Although no other individual radiological characteristic on USS, CT or MRI was predictive of MCN, MDT outcomes stating that a cyst was complex in nature were highly predictive (p = 0.0007). Cyst fluid carbohydrate antigen 19-9, carcino-embryonic antigen and cancer antigen 125 were unable to differentiate MCN from SC (p = 0.45, p = 0.49, and p = 0.73, respectively). CONCLUSIONS: MDT outcomes are of greatest value when trying to differentiate MCN from SC, as well as having a solitary cyst on imaging. Conventional cyst fluid tumour markers are unhelpful. All suspicious cystic liver lesions should be discussed pre-operatively by a hepatobiliary MDT to determine the most appropriate surgical approach.

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.

Delayed diagnosis of alpha-1-antitrypsin deficiency following post-hepatectomy liver failure: A case report.

Post-hepatectomy liver failure (PHLF) is a leading cause of morbidity and mortality following major liver resection. The development of PHLF is dependent on the volume of the remaining liver tissue and hepatocyte function. Without effective pre-operative assessment, patients with undiagnosed liver disease could be at increased risk of PHLF. We report a case of a 60-year-old male patient with PHLF secondary to undiagnosed alpha-1-antitrypsin deficiency (AATD) following major liver resection. He initially presented with acute large bowel obstruction secondary to a colorectal adenocarcinoma, which had metastasized to the liver. There was no significant past medical history apart from mild chronic obstructive pulmonary disease. After colonic surgery and liver directed neo-adjuvant chemotherapy, he underwent a laparoscopic partially extended right hepatectomy and radio-frequency ablation. Post-operatively he developed PHLF. The cause of PHLF remained unknown, prompting re-analysis of the histology, which showed evidence of AATD. He subsequently developed progressive liver dysfunction, portal hypertension, and eventually an extensive parastomal bleed, which led to his death; this was ultimately due to a combination of AATD and chemotherapy. This case highlights that formal testing for AATD in all patients with a known history of chronic obstructive pulmonary disease, heavy smoking, or strong family history could help prevent the development of PHLF in patients undergoing major liver resection.

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.

Simultaneous combined liver and kidney transplantation: a single center experience.

Renal dysfunction is common in patients awaiting liver transplantation (LT) and affects outcome following LT. Combined liver and kidney transplantation (CLKT) has been proposed as effective treatment for patients with chronic diseases of both organs, some with hepatorenal syndrome and for liver-based metabolic diseases affecting kidney. This study is undertaken to analyze results of CLKT at a single center. Of 2690 LTs performed between 1992 and 2007, there were 39 CLKTs; most common indications were metabolic, cirrhosis and polycystic disease. With follow-up of up to 170 months, 11 died (overall survival 71.8%); one-, five-, and 10-yr patient and liver graft survival is 77%, 73.7%, and 73.7%, respectively, and kidney graft survival is 77%, 70%, and 70%, respectively. Survival among metabolic group (78.6%) appeared to be better than non-metabolic group (68%); however, this difference was not significant (p = 0.39). Fifteen surviving patients (53.6%) have mild/moderate renal impairment (creatinine > or = 125 micromol/L). None has severe renal failure (serum creatinine > or = 250 micromol/L) or end-stage renal disease requiring hemodialysis. CLKT has good results in selected groups of patients. It provides protection to kidney allograft in liver-based metabolic diseases affecting kidney. The rate of acute rejection episodes of kidney is low. Significant proportion develops long-term mild/moderate renal dysfunction. Careful attention to immunosuppression to minimize nephrotoxicity may help.

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.

Pharmacokinetics, efficacy, and safety of mycophenolate mofetil in combination with standard-dose or reduced-dose tacrolimus in liver transplant recipients.

The pharmacokinetics of mycophenolate mofetil (MMF) in liver transplant recipients may change because of pharmacokinetic interactions with coadministered immunosuppressants or because changes in the enterohepatic anatomy may affect biotransformation of MMF to mycophenolic acid (MPA) and enterohepatic recirculation of MPA through the hydrolysis of mycophenolate acid glucuronide to MPA in the gut. In the latter case, the choice of formulation (oral versus intravenous) could have important clinical implications. We randomized liver transplant patients (n = 60) to standard (10-15 ng/mL) or reduced (5-8 ng/mL) trough levels of tacrolimus plus intravenous MMF followed by oral MMF (1 g twice daily) with corticosteroids. Pharmacokinetic sampling was performed after the last intravenous MMF dose, after the first oral MMF dose, and at selected times over 52 weeks. The efficacy and safety of the 2 regimens were also assessed. Twenty-eight and 27 patients in the tacrolimus standard-dose and reduced-dose groups, respectively, were evaluated. No significant differences between the tacrolimus standard-dose and reduced-dose groups were seen in dose-normalized MPA values of the time to the maximum plasma concentration (1.25 versus 1.28 hours), the maximum plasma concentration (15.5 +/- 7.93 versus 13.6 +/- 7.03 microg/mL), or the area under the concentration-time curve from 0 to 12 hours (AUC(0-12); 53.0 +/- 20.6 versus 43.8 +/- 15.5 microg h/mL) at week 26 or at any other time point. No relationship was observed between the tacrolimus trough or AUC(0-12) and MPA AUC(0-12). Exposure to MPA after oral and intravenous administration was similar. Safety and efficacy were similar in the two treatment groups. In conclusion, exposure to MPA is not a function of exposure to tacrolimus. The similar safety and efficacy seen with MMF plus standard or reduced doses of tacrolimus suggest that MMF could be combined with reduced doses of tacrolimus.

Spontaneous rupture of hepatocellular carcinoma: a Western experience.

BACKGROUND: Spontaneous rupture of hepatocellular carcinoma (HCC) is a life-threatening presentation, with an incidence of <3% of HCC patients in Western countries. The reported overall mortality is < or =50% in Asian countries, where the incidence is 12% to 14%. The aim of this study was to report a single center's experience of patients with ruptured HCC during a 11-year period. METHODS: A retrospective review was performed of all patients who presented with ruptured HCC between 1995 and 2005. Data on clinical features, treatment strategies, and survival outcomes were collected. Statistical methods included univariate analysis and Kaplan-Meier survival estimates with log-rank test. RESULTS: A cohort of 21 patients (15 male and 6 female) was identified. Fourteen (66.6%) patients had histologic evidence of underlying cirrhosis, ad the median age at presentation was 68 years (interquartile range [IQR] 61 to 69). Ten of these patients (71.4%) were hemodynamically unstable at presentation. The mean tumor size was 8.5 cm (range 3 to 13), and there was multifocal disease in 6 (42.8%) patients. The etiology of cirrhosis was hepatitis B infection in 3, hepatitis C in 3, alcohol in 4, and cryptogenic in 4 patients. Initial bleeding control was attempted by transarterial embolization (TAE) in 7 (50%) and by emergency surgery in 7 patients (50%). Four of the operations were performed at referring hospitals, and 3 were performed at our institution. Two patients (14.2%) underwent palliative treatment only. Definitive treatment included resection at emergency surgery in 1, staged hepatectomy in 1, and transarterial chemoembolization in 2 patients. There were 7 patients who were noncirrhotic and had a median age of 51 years (IQR 42 to 60). Of these, 6 (87.5%) were hemodynamically unstable at presentation. Mean tumor size was 9 cm (range 6 to 18) and confined to right lobe in all patients. Primary hemostasis was successfully achieved by TAE in 2 and perihepatic packing in 1 patient. Definitive treatment was provided by emergency hepatectomy in 4 and staged hepatectomy in 3 patients. Patients with cirrhosis (n = 14) had a median survival rate of <30 days. Child-Pugh score at presentation (median 7, IQR 5 to 8) correlated strongly with overall survival (P <.0001). Median survival for noncirrhotic patients was 20 months (IQR 2 to 31). One patient without cirrhosis survived for 122 months without disease recurrence. CONCLUSIONS: Spontaneous rupture of HCC is an uncommon presentation in Western countries. Primary hemostasis, followed by emergency or staged hepatic resection, is the treatment of choice. Median survival in patients initially treated with surgery was better than that observed in patients who underwent initial TAE, although this was not statistically significant. Patients who had no underlying liver disease had better prognosis than those who had cirrhosis.