Inflammatory myofibroblastic tumours of the liver - a systematic review.

BACKGROUND: Hepatic inflammatory myofibroblastic tumours (HIMTs) are rare and poorly described in the literature. Most publications are single patient case reports and lack detailed reporting on characteristics, management, and outcomes. This systematic review aimed to assess the demography, clinical presentation, typical imaging features, histopathology, treatment, and outcomes of patients presenting with HIMTs. METHODS: A systematic literature search was performed in MEDLINE (PubMed), EMBASE (Scopus), JSTOR, Cochrane CENTRAL (Cochrane Library), and the databases included in the Web of Science for studies published between 1940 and 2023 on HIMTs, including its reported synonyms. Case series or cohort studies that reported on the management and outcomes of at least four patients with histologically confirmed HIMTs were included in the analysis. RESULTS: After screening 4553 publications, 22 articles including a total of 440 patients with confirmed HIMTs were eligible for inclusion. The average age was 53.4 years (range 42.0-65.0) with a male to female ratio of 1.7:1. Abdominal pain, discomfort, fever, and loss of weight were the most common presenting symptoms. Surgical resection is the standard of care for HIMTs and is associated with low mortality of 3.4% and low disease recurrence. CONCLUSION: HIMT is a disease more often affecting middle-aged males. The lesions are typically solitary with low recurrence after treatment. The relative roles of surgical versus medical treatment remain unclear. Differences in clinical presentation, histopathology, and treatment of HIMTs compared to inflammatory myofibroblastic tumour (IMT) at extrahepatic sites could challenge the current view of IMT as a single pathological entity.

Does the textbook outcome in pancreatic surgery score after pancreaticoduodenectomy for ampullary carcinoma have prognostic value?

BACKGROUND: The value of the textbook outcome in pancreatic surgery (TOPS) score, a composite measure of surgical performance for quality assurance, was evaluated in a South African tertiary hospital cohort of pancreaticoduodenectomies (PD) performed for adenocarcinoma of the ampulla of Vater (AAV). METHODS: A review of all patients undergoing a PD for AAV at a single centre between January 1999 and December 2023 was performed. Demographic, operative, pathological and postoperative variables were recorded. Ten clinical and histological variables were used to construct a TOPS score. These included an R0 resection, no postoperative pancreatic fistula (POPF), no bile leak, no post-pancreatectomy haemorrhage, no delayed gastric emptying, no major postoperative complications (< Gr 3 Clavien-Dindo), no readmission to ICU, length of stay ≤ 10 days, no 30-day readmission or intervention and no 30-day mortality. A textbook outcome (TO) was defined as the fulfilment of all 10 variables. In patients in whom TO was not achieved, the reasons for failure were identified. In addition, the number of patients who had major complications and died were categorised as failure to rescue (FTR). RESULTS: A positive TOPS score was achieved in 27 of 79 (34.2%) patients undergoing a PD. Overall five-year survival after PD was 33.9%. TOPS conferred a significant 1-year survival benefit, 88.9% vs 66.7% (OR 4.12, 95% CI 1.08-15.67, p = 0.038). There was no significant difference in 5-year survival between TOPS and non-TOPS patients, 40.0% vs 32.4% (OR 1.39, 95% CI 0.48-3.99, p = 0.54). A POPF occurred in 31.6% patients, resulting in a significantly longer hospital admission, 17 vs 10 days (95% CI 2.66-11.34, p = 0.0019). Twenty-one (26.6%) patients developed a major complication, five of whom died (FTR = 6.3%). CONCLUSION: This study confirmed the value of TOPS as a useful measurement to assess hospital quality metrics and short-term survival after PD for AAV. One quarter of patients developed a major complication with a 6.3% FTR.

Liver resection for hepatocellular and fibrolamellar carcinoma in a South African tertiary referral centre - an observational cohort analysis.

BACKGROUND: More than 80% of global hepatocellular carcinomas (HCC) occur in sub-Saharan Africa (SSA) and South- East Asia. Compared with the rest of the world, HCC in SSA has the lowest resection and survival rates. This study assessed outcome following liver resection for HCC and fibrolamellar carcinoma (FLC) at a tertiary referral centre in South Africa. METHODS: A retrospective analysis was done of all liver resections for HCC and FLC at Groote Schuur Hospital and the University of Cape Town Private Academic Hospital between January 1990 and December 2021. Three groups were compared, (i) HCC occurring in normal livers, (ii) HCC occurring in cirrhotic livers, and (iii) fibrolamellar carcinoma. Postoperative complications were classified as per the expanded accordion severity grading system. Median overall survival (OS) and 95% confidence intervals (CI) were calculated. RESULTS: Forty-eight patients were included in the study, 25 for HCC in non-cirrhotic livers, 15 in cirrhotic livers and eight for FLC. Thirty-six patients (75%) underwent a major resection. No mortality occurred but 16 patients (33%) developed grade 1 to 4 complications postoperatively. Thirty-three patients (69%) developed recurrence of HCC following their initial resection of whom 29 (60%) ultimately died. Median overall survival (OS) for the total cohort after surgery was 57.2 months, 95% CI (29.7-84.6), 64.2 months (29.7-84.6), 61.9 months (28.1-95.6), and 31.7 months (1.5-61.8) for patients with HCC in non-cirrhotic livers, FLC and HCC in cirrhotic livers respectively. CONCLUSION: Liver resection for HCC and FLC was safe with no mortality, but one-third of patients had associated postoperative morbidity. The high long-term recurrence rate remains a major obstacle in achieving better survival results after resection.

Effect of preoperative biliary drainage on intraoperative biliary cultures and surgical outcomes after pancreatic resection.

BACKGROUND: Prolonged obstructive jaundice (OJ), associated with resectable pancreatic pathology, has many deleterious effects that are potentially rectifiable by preoperative biliary drainage (POBD) at the cost of increased postoperative infective complications. The aim of this study is to assess the impact of POBD on intraoperative biliary cultures (IBCs) and surgical outcomes in patients undergoing pancreatic resection. METHODS: Data from patients at Groote Schuur Hospital, Cape Town, between October 2008 and May 2019 were analysed. Demographic, clinical, and outcome variables were evaluated, including perioperative morbidity, mortality, and 5-year survival. RESULTS: Among 128 patients, 69.5% underwent POBD. The overall perioperative mortality in this study was 8.8%. The POBD group had a significantly lower perioperative mortality rate compared to the non-drainage group (5.6% vs. 25.6%). POBD patients had a higher incidence of surgical site infections (55.1% vs. 23.1%), polymicrobial growth from IBCs and were more likely to culture resistant organisms. Five-year survival was similar in the two groups. CONCLUSION: POBD was associated with a high incidence of resistant organisms on the IBCs, a high incidence of surgical site infections and a high correlation between cultures from the surgical site infection and the IBCs.

Intermediate and long-term survival prediction using prognostic scores in patients undergoing salvage TIPS for uncontrolled variceal bleeding.

BACKGROUND: This study investigated the value of prognostic scores to predict 90-day, 1-, 3- and 5-year survival after salvage TIPS (sTIPS) in patients with exsanguinating variceal bleeding who failed endoscopic intervention. METHODS: The Model for End-Stage Liver Disease (MELD), Model for End-Stage Liver Disease Sodium (MELDNa), Acute Physiology and Chronic Health Evaluation II (APACHE II) and Child-Pugh (C-P) grades and scores were calculated using Kaplan-Meier curves and Cox proportional hazards models in sTIPS patients treated between August 1991 and November 2020. RESULTS: Thirty-four patients (29 men, 5 women), mean age 52 years, SD ± 11.6 underwent sTIPS which controlled bleeding in 32 (94%) patients. Ten (29.4%) patients died in hospital at a median of 4.8 (range 1-10) days. On bivariate analysis, C-P score ≥ 10 (p = 0.017), high C-P grade (p = 0.048), MELD ≥ 15 (p = 0.010), MELD-Na score ≥ 22 (p < 0.001) and APACHE II score ≥ 15 (p < 0.001) predicted 90-day mortality. Individual clinical characteristics associated with 90-day mortality were grade 3 ascites (p = 0.029), > 10 units of blood transfused (p = 0.004), balloon tube placement (p < 0.001), endotracheal intubation (< 0.001) and inotrope support (p < 0.001). The overall 90-day, 1-, 3- and 5-year survival rates were 67.6%, 55.9%, 26.5% and 20.6% respectively. Nine patients (26.5%) were alive at a median of two years (range 1-18 years) post-TIPS. Patients with C-P grade A, C-P score < 10, MELD score < 15, MELD-Na score < 22 and APACHE II score < 15 had significantly better 90-day, 1-, 3- and 5-year survival rates. CONCLUSION: Although sTIPS controlled variceal bleeding in 94% of patients after failed endoscopic therapy, in-hospital mortality was 29% and less than one quarter were alive after five years. The selected cut-off values for the nominated scoring systems accurately predicted 90-day mortality and long-term survival.

Factors influencing in-hospital mortality for salvage percutaneous transjugular intrahepatic portosystemic shunting in cirrhotic patients with recalcitrant variceal bleeding after failed endoscopic intervention.

BACKGROUND: Endoscopic therapy is the first-line treatment of choice for control of acute variceal bleeding (AVB). In high-risk patients with persistent AVB despite pharmacological treatment and endoscopic intervention, percutaneous transjugular intrahepatic portosystemic shunting (TIPS) provides a minimally invasive salvage method to reduce portal pressure and control bleeding. OBJECTIVES: To evaluate factors influencing in-hospital mortality after salvage TIPS (sTIPS) in patients with exsanguinating variceal bleeding despite medical treatment and endoscopic intervention. METHODS: Clinical and laboratory data were analysed in all patients treated with sTIPS following failed endoscopic therapy for AVB between August 1991 and November 2020. Factors associated with and predictors of death were determined using bivariate analysis and univariate logistic regression analysis. RESULTS: Thirty-four patients (29 men, 5 women), mean age 52 years (range 31 - 80), received sTIPS for uncontrolled (n=11) or refractory (n=23) AVB. The causes of portal hypertension were alcohol-related (n=24) and non-alcohol-related cirrhosis. Salvage TIPS controlled bleeding in 32 patients, with recurrence in 1. Ten patients died in hospital (mean 4.8 days, range 1 - 10) of liver failure (n=4), multiorgan failure (n=3), alcoholic cardiomyopathy (n=2) and uncontrolled gastric variceal bleeding (n=1). On bivariate analysis, factors associated with death were Child-Pugh (C-P) score ≥10 (p=0.006), sodium Model for End-stage Liver Disease (MELD-Na) score ≥22 (p<0.001), ≥8 units of blood transfused (p<0.001), Sengstaken-Blakemore balloon tube placement (p<0.001), endotracheal intubation (p<0.001), inotropic support (p<0.001) and endoscopically uncontrolled bleeding (p<0.001). Univariate logistic regression analysis showed that the most significant predictors of mortality were inotrope dependency (odds ratio (OR) 134; p<0.001), endotracheal intubation (OR 99; p<0.001), endoscopically uncontrolled bleeding (OR 28; p=0.001), grade 3 ascites (OR 20.9; p=0.012) and C-P grade C (OR 8.8; p=0.011). CONCLUSION: Salvage TIPS controlled variceal bleeding in 94% of patients after failed endoscopic therapy with 29% in-hospital mortality. The most significant predictors of mortality were C-P grade C, grade 3 ascites, inotrope requirement, endotracheal intubation and endoscopically uncontrolled bleeding.

Trans-arterial therapy for Fibrolamellar carcinoma: A case report and literature review.

INTRODUCTION: Fibrolamellar carcinoma (FLC) is a rare pathologically distinct primary liver cancer. Surgical resection is the only treatment associated with prolonged survival. Trans-arterial embolization (TAE), which is a recognised treatment for hepatocellular carcinoma has been used to treat FLC. We present a case and performed a literature review of patients with FLC treated with TAE. CASE PRESENTATION: We present a 19-year old female with a large potentially resectable FLC which was initially treated with trans-arterial chemo-embolization (TACE) with drug eluting beads. The TACE was followed by surgical resection. Histology confirmed tumour necrosis related to the previous TACE. DISCUSSION & LITERATURE REVIEW: We identified seven case reports and one case series of TAE for FLC. TAE was either used as a neo-adjuvant therapy to facilitate subsequent tumour resection or as a palliative treatment modality. We propose an algorithm for the treatment of FLC that includes TAE. CONCLUSION: The rarity of FLC and the paucity of data precludes establishing clear evidence-based standards of care. We propose an algorithm for the treatment of FLC. The establishment of an international registry may facilitate the collection of better quality evidence.

Resection of biliary mucinous cystic neoplasms of the liver: a prospective cohort series of 13 consecutive patients.

BACKGROUND: Biliary mucinous cystic neoplasms (BMCNs) are uncommon neoplastic septated intrahepatic cysts which are often incorrectly diagnosed and have the potential for malignant transformation. OBJECTIVE: To assess the outcome of surgical resection of BMCNs. METHOD: A prospective liver surgery database was used to identify patients who underwent surgery at Groote Schuur Hospital Complex for BMCN from 1999 to 2015. Demographic variables including age and gender were documented as well as detailed preoperative imaging, location and size, operative treatment, extent of resection, histology, postoperative complications and outcome. RESULTS: Thirteen female patients (median age 45 years) had surgery. Eleven were diagnosed by imaging for symptoms. Two were jaundiced. One cyst was found during an elective cholecystectomy. Five cysts were located centrally in the liver. Before referral three cysts were treated with percutaneous drainage and two were treated with operative deroofing. Six patients had anatomical liver resections and seven patients had non anatomical liver resections of which two needed ablation of residual cyst wall. One patient needed a biliary-enteric reconstruction to treat a fistula. Median operative time was 183 minutes (range: 130-375). No invasive carcinoma was found. There was no operative mortality. One surgical site infection and one intra-abdominal collection were treated. Two patients developed recurrent BMCN after 24 months. CONCLUSION: BMCNs should be considered in middle aged women who have well encapsulated multilocular liver cysts. Treatment of large central BMCNs adjacent to vascular and biliary structures may require technically complex liver resections and are best managed in a specialised hepato-pancreatico-biliary unit.

Distal malignant biliary obstruction: a prospective randomised trial comparing plastic and uncovered self-expanding metal stents in the palliation of symptomatic jaundice.

BACKGROUND: The aim of this study was to determine the safety and clinical effectiveness of 10Fr plastic biliary stents compared to uncovered self-expanding metal stents (SEMS) for palliative treatment of patients with inoperable extra-hepatic malignant biliary obstruction in a public hospital in South Africa. METHOD: From January 2009 to December 2013, 40 patients who were admitted to a tertiary academic centre because of distal malignant biliary obstruction were enrolled in a prospective randomized study. Patients were randomly assigned to receive an uncovered SEMS or a plastic stent deployed through the biliary stricture during endoscopic retrograde cholangiopancreatography (ERCP). RESULTS: Patient survival time in the two groups did not differ significantly (median: SEMS - 114 days; plastic - 107 days). Stent failure was more common in the plastic stent group (7/19 vs. 1/21). The results became significant after 6 months of follow-up. There was no significant difference between the two groups in the incidence of serious adverse events. CONCLUSION: SEMS had a longer duration of patency than plastic stents, which recommends their use in the palliative treatment of patients with biliary obstruction due to distal malignant biliary obstruction.

Distal malignant biliary obstruction: a prospective randomised trial comparing plastic and uncovered self-expanding metal stents in the palliation of symptomatic jaundice

Background: The aim of this study was to determine the safety and clinical effectiveness of 10Fr plastic biliary stents compared to uncovered self-expanding metal stents (SEMS) for palliative treatment of patients with inoperable extra-hepatic malignant biliary obstruction in a public hospital in South Africa.Methods: From January 2009 to December 2013, 40 patients who were admitted to a tertiary academic centre because of distal malignant biliary obstruction were enrolled in a prospective randomized study. Patients were randomly assigned to receive an uncovered SEMS or a plastic stent deployed through the biliary stricture during endoscopic retrograde cholangiopancreatography (ERCP).Results: Patient survival time in the two groups did not differ significantly (median: SEMS ­ 114 days; plastic ­ 107 days). Stent failure was more common in the plastic stent group (7/19 vs. 1/21). The results became significant after 6 months of follow-up. There was no significant difference between the two groups in the incidence of serious adverse events.Conclusions: SEMS had a longer duration of patency than plastic stents, which recommends their use in the palliative treatment of patients with biliary obstruction due to distal malignant biliary obstruction

Resection of biliary mucinous cystic neoplasms of the liver: a prospective cohort series of 13 consecutive patients

Background: Biliary mucinous cystic neoplasms (BMCNs) are uncommon neoplastic septated intrahepatic cysts which are often incorrectly diagnosed and have the potential for malignant transformation.Objectives:To assess the outcome of surgical resection of BMCNs.Methods:A prospective liver surgery database was used to identify patients who underwent surgery at Groote Schuur Hospital Complex for BMCN from 1999 to 2015. Demographic variables including age and gender were documented as well as detailed preoperative imaging, location and size, operative treatment, extent of resection, histology, postoperative complications and outcome.Results:Thirteen female patients (median age 45 years) had surgery. Eleven were diagnosed by imaging for symptoms. Two were jaundiced. One cyst was found during an elective cholecystectomy. Five cysts were located centrally in the liver. Before referral three cysts were treated with percutaneous drainage and two were treated with operative deroofing. Six patients had anatomical liver resections and seven patients had non anatomical liver resections of which two needed ablation of residual cyst wall. One patient needed a biliary-enteric reconstruction to treat a fistula. Median operative time was 183 minutes (range: 130­375). No invasive carcinoma was found. There was no operative mortality. One surgical site infection and one intra-abdominal collection were treated. Two patients developed recurrent BMCN after 24 months.Conclusion:BMCNs should be considered in middle aged women who have well encapsulated multilocular liver cysts. Treatment of large central BMCNs adjacent to vascular and biliary structures may require technically complex liver resections and are best managed in a specialised hepato-pancreatico-biliary unit

Outcome of liver resection for small bowel neuroendocrine tumour metastases

Background: Small bowel neuroendocrine tumours frequently metastasise to the liver. While liver resection improves survival and provides symptomatic relief, multifocal bilobar disease adds complexity to surgical management.Objectives: This study evaluated outcome in patients with small bowel neuroendocrine liver metastases who underwent liver resection at Groote Schuur Hospital and UCT Private Academic Hospital.Methods: All patients with small bowel neuroendocrine liver metastases treated with resection from 1990­2015 were identified from a prospective departmental database. Demographic data, operative management, morbidity and mortality using the Accordion classification were analysed. Survival was assessed using the Kaplan-Meier method.Results: Seventeen patients (9 women, 8 men, median age 55 years, range 31­76) underwent resection. Each patient had all identifiable liver metastases resected and/or ablated (median n = 3, range 1­20). Ten patients had major anatomical liver resections. Three patients had five segments resected, and seven had four resected. Nine patients (53%) had a concurrent bowel resection of the small bowel NET primary and a regional mesenteric lymphadenectomy. Median operating time was 255 min (range 150­720). Median blood-loss was 800 ml (range 200­10,000). Five patients required intraoperative blood transfusion. Hepatic vascular inflow control was used in ten patients (56.5 min median, range 20­150 min), which included hepatic inflow control n = 8, total hepatic exclusion n = 1, and selective hepatic exclusion n = 1. Median postoperative hospital stay was 9 days (range 2­28). Thirteen complications occurred in seven patients. Accordion grades were 1 n = 3, 2 n = 4, 3 n = 3, 4 n = 2, 6 n = 1. One patient required reoperation for bleeding and a bile leak. One patient died of a myocardial infarction 36 hours postoperatively. Sixteen patients (94%) had symptomatic improvement. Five-year overall survival was 91% (median follow-up 36 months, range 14­86 months).Conclusion: Our data show that liver resection can be safely performed for small bowel NET metastases with a good 5-year survival. However, a substantial number of patients require a major liver resection and these patients are best managed at a multidisciplinary referral centre

Incidence and management of postoperative bile leaks: A prospective cohort analysis of 467 liver resections.

BACKGROUND: Bile leaks from the parenchymal transection margin are a major cause of morbidity following major liver resections. The aim of this study was to benchmark the incidence and identify the risk factors for postoperative bile leakage after hepatic resection. PATIENTS AND METHODS: A prospective database of 467 consecutive liver resections performed by the University of Cape Town HPB surgical unit between January 1990 and January 2016 was analysed. The relationship of demographic, clinical and perioperative factors to the development of bile leakage was determined. Bile leak and postoperative complications severity were graded using the International Study Group of Liver Surgery and Accordion classifications. RESULTS: Overall morbidity was 24% (n = 112), with bile leaks occurring in 25 (5.4%) patients. Significantly more bile leaks occurred in patients who had major resections (≥ 3 segments) and longer total operative times (p < 0.05). There were 5 Grade A bile leaks which stopped spontaneously. Seventeen Grade B leaks required a combination of percutaneous drainage (n = 15), endoscopic biliary stenting (n = 8) and percutaneous transhepatic biliary drainage (n = 3). All 3 Grade C leaks required laparotomy for definitive drainage. Median hospital stay in the 442 patients without a bile leak was 8 days (IQR 1-98) compared with 12 days (IQR 6-30) for the 25 with bile leaks (p < 0.05) with no mortality. Major resections (≥ 3 segments) and total operative time (> 180mins) were significantly associated with bile leaks. CONCLUSION: The incidence of bile leakage was 5.4% and occurred after major liver resections with longer operative times and resulted in significantly extended hospitalisation. Most were effectively treated nonoperatively by percutaneous drainage of the collection and/or endoscopic or percutaneous biliary drainage without mortality.

Severe post-pancreatoduodenectomy haemorrhage: An analytical review based on 118 consecutive pancreatoduodenectomy patients in a South African Academic Hospital.

BACKGROUND: Bleeding after a major pancreatic resection, although uncommon, has serious implications and substantial mortality rates. AIM: To analyse our experience with severe post-pancreatoduodenectomy haemorrhage (PPH) over the last 7 years to establish the incidence, causes, intervention required and outcome. METHOD: All patients who underwent a pancreatoduodenectomy (PD) between January 2008 and December 2015 were identified from a prospectively maintained database. Data analysed included demographic information, operative details, anastomotic technique, histology, postoperative complications including pancreatic fistula and PPH, length of hospital stay, need for blood products and special investigations. Pancreatic fistula was classified according to the International Study Group of Pancreatic Surgery (ISGPS) classification. A modified ISGPS classification was used for PPH. RESULTS: One hundred and eighteen patients underwent PD during the study period of whom 6 (5.0%) died perioperatively. Twenty patients (16.9%) developed a pancreatic fistula and 11 patients (9.3%) had a severe PPH of whom one (9.1%) died. No patients had a severe bleed during the first 24 hours postoperatively. Four patients bled within the first 5 days and the remaining 7 after five days. Six patients bled from the gastroduodenal artery and were all preceded by a pancreatic fistula. Three of the 7 patients who bled late presented with extraluminal bleeding, 3 presented with intraluminal bleeding and 1 with a combination of both. Patients presenting in the first 5 days were all successfully managed either endoscopically or surgically. Five patients who presented beyond 5 days postoperatively were managed primarily with interventional angiography, either with coiling or deployment of a covered stent. Three patients who had radiological intervention developed a liver abscess or necrosis. CONCLUSION: Severe PPH is associated with substantial morbidity. Clinical factors including the onset of the bleeding, presentation with either extra and/or intraluminal haemorrhage, and the presence of a pancreatic fistula give an indication of the likely aetiology of the bleeding. A management algorithm based on these factors is presented.