Epithelioid hemangioendothelioma of the liver as a rare indication for liver transplantation

AIM: To investigate the indications and outcomes of liver transplantation for hepatic epithelioid hemangioendothelioma(HEHE). METHODS: Between 1989 and August 2013, in the Department of General, Transplant, and Liver Surgery, Medical University of Warsaw, 1306 orthotopic liver transplantations(OLTx) were performed, including 72 retransplantations. Unresectable HEHE was an indication for OLTx in 10 patients(0.8% of primary OLTx), the mean age of the patients was 40.5 ± 13.3 years(range 23-65 years), and the male-to-female ratio was 2:8. Kaplan-Meier survival analysis in HEHE, hepatocellular carcinoma(HCC), and other OLTx recipients groups was performed. The differences in mortality were compared using the χ2 test. A P-value < 0.05 indicated statistical significance.RESULTS: No concomitant liver disease was found in any patient. There was no neoadjuvant chemotherapy or radiotherapy. Liver function test results were normal in most of the patients. The levels of alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 were normal. In immunohistochemical staining, the neoplastic cells were positive for factor Ⅷ-related antigen, CD31, and CD34, which are endothelial cell markers, and negative for cytokeratin 19, cytokeratin 7, and HepPar-1. Nine patients were alive without tumor recurrence. One patient died 2 mo after OLTx due to septic complications. No morbidity was observed. Maximum follow-up was 11.4 years, with a minimum of 1 mo. The cumulative survival rate at the end of followup in HEHE patients was 87.5% compared with 54.3% in the HCC group and 76.3% in the other OLTx recipients group(χ2 test = 1.784, df = 2, P = 0.409).CONCLUSION: Unresectable HEHE, without extrahepatic metastases is an excellent indication for liver transplantation. Long-term survival is very good and much better than in HCC patients and the entire group of OLTx patients.

Isolated right posterior bile duct injury following cholecystectomy:Report of two cases

Anatomic variations of the right biliary system are one of the most common risk factors for sectoral bile duct injury(BDI)during cholecystectomy.Isolated right posterior BDI may in particular be a challenge for both diagnosis and management.Herein we describe two cases of isolated right posterior sectoral BDI that took place during laparoscopic cholecystectomy.Despite effective external biliary drainage from the liver hilum in both cases,there was a persistent biliary leak observed which was not visible on endoscopic retrogradecholangiogram.Careful evaluation of images from both endoscopic and magnetic resonance cholangiograms revealed the diagnosis of an isolated right posterior sectoral BDI.These were treated with a delayed bisegmental(segments 6 and 7)liver resection and a Roux-en-Y hepaticojejunostomy respectively with good outcomes at 24 and 4 mo of follow-up.This paper discusses strategies for prevention of such injuries along with the diagnostic and therapeutic challenges it offers.

Fulminant liver failure following a marathon: Five case reports and review of literature

BACKGROUND The growing popularity of marathon and half-marathon runs has led to an increased number of patients presenting with exertion-induced heat stroke. Mild hepatic involvement is often observed in these patients;however, fulminant liver failure may occur in approximately 5% of all cases. Liver transplantation is a potentially curative approach for exertion-induced liver failure, although there is a lack of consensus regarding the criteria and optimal timing of this intervention. CASE SUMMARY This paper describes 5 patients (4 men and 1 woman) who were referred to the department where this study was performed with the diagnosis of exertioninduced acute liver failure. Three patients underwent liver transplantation, 1 recovered spontaneously, and 1 patient died on day 11 following the exertion. CONCLUSION Exertion-induced heat stroke may present as fulminant liver failure. These patients may recover with conservative treatment, may require liver transplantation, or may die. No definitive criteria are available to determine patient suitability for a conservative vs surgical approach.

Spontaneous rupture of a mucinous cystic neoplasm of the liver resulting in a huge biloma in a pregnant woman:A case report

BACKGROUND Mucinous cystic neoplasm of the liver(MCN-L)and intraductal papillary neoplasm of the bile duct(IPN-B)are two different types of mucin-producing bile duct tumour that may complicate the course of pregnancy.To the best of our knowledge,we describe herein the first case of MCN-L with spontaneous rupture during pregnancy necessitating complex surgical treatment.CASE SUMMARY A 24-year-old woman was initially admitted to another hospital in October 2018 with signs of jaundice(serum bilirubin level 12 mg/dL)and upper abdominal pain radiating to the left shoulder.Initial magnetic resonance imaging(MRI)of the abdominal cavity revealed a multilocular cystic tumour of the liver hilum(37 mm×40 mm in diameter)located between segments 3 and 4 of the left liver lobe.Six weeks later(December 2018),the patient was found to be 12 wk pregnant and was referred to our institution for further diagnostics and treatment.At admission,a soft,palpable,and tender mass in the left upper abdomen was found.It was determined via MRI(with no intravenous contrast in view of the first-trimester pregnancy)to be a large collection of fluid(19 cm×17 cm×10 cm)located close to the liver hilum and below the left liver lobe.The patient did not undergo any diagnostic or therapeutic procedures nor did they have any abdominal trauma in the preceding weeks.The fluid collection proved to be of biliary origin following percutaneous drainage.Therefore,we concluded this was a spontaneous rupture of an MCN-L with the formation of a biloma.The MRI study also revealed the previously found cystic tumour of the liver hilum communicating with the left hepatic duct,which,together with left hepatic duct dilatation,suggested the diagnosis of IPN-B.The follow-up MRI with intravenous gadolinium contrast performed in the second trimester of pregnancy(week 14)showed,in turn,some features of MCN-L,including enhancement of the internal septations within the cystic liver mass.A precise preoperative differential diagnosis between IPN-B and MCN-L was therefore not possible.The patient was submitted to surgery in the second trimester of pregnancy(week 18).Surgery included a cholecystectomy,left hepatectomy,and concomitant resection of the extrahepatic bile ducts followed by anastomosis of the right hepatic duct with the Roux limb of the jejunum.The post-operative period was uneventful and the patient was discharged 8 days after surgery.The histopathological examination of the resected specimen revealed a final diagnosis of MCN-L with low-grade dysplasia and epithelium surrounded by ovarian-type stromal tissue.The patient delivered a healthy baby girl and both remain well at present,after 2 years of follow-up since surgery.CONCLUSION The differential diagnosis and management of MCN-L and IPN-B may be very challenging,particularly in the setting of pregnancy.When indications for surgery are obvious,the final diagnosis is based on histopathological examination,with ovarian-type stroma being pathognomonic for MCN-L.We believe that the growth of this subepithelial stroma secondary to the high levels of sex hormones produced during pregnancy might have been the main causative factor leading to the tumour rupture with the formation of a biloma in our patient.