Risk for Recurrence in Long-Term Follow-Up of Children after Liver Transplantation for Hepatoblastoma or Hepatocellular Carcinoma.

The aim of this study was to assess the long-term results of liver transplantation (LT) in pediatric patients with unresectable hepatoblastoma (HB) or hepatocellular carcinoma (HCC) with special reference to the risk of tumor recurrence. We retrospectively analyzed data from 46 HB and 26 HCC patients who underwent LT between 1990 and 2022. In HCC patients, we compared outcomes depending on donor type. We evaluated the impact of a number of risk factors on recurrence-free survival after LT. Estimated patient survival after 5, 10, and 15 years was 82%, 73%, and 73% in the HB group and 79%, 75%, and 75% in the HCC group, respectively (p = 0.76). In the HCC group, living donor LT (LDLT) and deceased donor LT (DDLT) provided similar patient survival (p = 0.09). Estimated recurrence-free survival in patients who had three or fewer risk factors was significantly better than in patients with more than three risk factors (p = 0.0001). Adequate patient selection is necessary when considering LT for primary liver tumors in children. The presence of more than three risk factors is associated with a very high risk of recurrence and indicates poor prognosis, whereas extrahepatic disease may be considered a contraindication for transplantation.

Is M30 staining a reliable marker predicting graft rejection and fibrosis in protocolar liver biopsies in post-liver transplant pediatric patients?

BACKGROUND: Liver transplantation is currently a treatment of choice in patients with end-stage liver disease. Acute cellular rejection (ACR), antibody-mediated rejection (AMR), and chronic rejection (ChR) are major causes of graft injury. Therefore, new markers predicting graft rejection are investigating. Apoptosis has been recently proposed as one of the mechanisms contributing to liver fibrosis in liver grafts. Coarse needle liver biopsy is still a gold standard in monitoring post-transplant pathologies. The aim of this study was to assess the utility of immunohistochemical (IHC) staining for M30 (cytokeratin 18), as a prognostic marker of rejection in pediatric recipients of liver transplant and predicting marker of liver fibrosis and worse follow-up. METHODS: The study enrolled 55 biopsies from 55 patients aged 2.37 to 18.9 years (median 13.87 years) who underwent protocolar liver biopsies taken 1-17 years after liver transplantation (median 8.36 years). The control group (positive control group) consisted of 26 biopsies from 16 patients in whom acute ACR was diagnosed. IHC staining for M30 (cytokeratin 18) and histochemical Azan staining were performed in all liver specimens. The following changes were re-evaluated in each specimen: features of ACR (the severity was assessed using RAI/Rejection Activity Index/Scale, which ranges from 3-9 points and include 3 histopathological changes suggestive of rejection), AMR or ChR; severity of fibrosis (Ishak Scale); presence of cholestasis and steatosis. Clinical parameters including laboratory tests of liver function (AST, ALT, GGTP, bilirubin) were also evaluated. RESULTS: M30 expression correlated with presence of acute cellular rejection. However, no relationship was found between M30 expression and severity of fibrosis. CONCLUSIONS: M30 staining, marker of apoptosis, seems to be a promising marker predicting acute cellular rejection.

The Impact of Hepatic Artery Thrombosis on the Outcome of Pediatric Living Donor Liver Transplantations.

The aim of our study was to assess risk factors for hepatic artery thrombosis (HAT) and to evaluate the impact of HAT management on long-term outcomes after pediatric living donor liver transplantation (LDLT). We retrospectively analyzed 400 patients who underwent primary LDLT between 1999 and 2020. We compared preoperative data, surgical factors, complications, and patient and graft survivals in patients with HAT (HAT Group) and without HAT (non-HAT Group). A total of 27 patients (6.75%) developed HAT. Acute liver failure, a hepatic artery (HA) anastomosis diameter below 2 mm, and intraoperative HA flow dysfunction were significantly more common in the HAT Group (p < 0.05, p = 0.02026, and p = 0.0019, respectively). In the HAT Group, 21 patients (77.8%) underwent urgent surgical revision. The incidence of biliary stenosis and retransplantation was significantly higher in the HAT Group (p = 0.00002 and p < 0.0001, respectively). Patient and graft survivals were significantly worse in the HAT Group (p < 0.05). The close monitoring of HA flow with Doppler ultrasound during the critical period of 2 to 3 weeks after LDLT and the immediate attempt of surgical revascularization may attenuate the elevated risk of biliary stenosis, graft loss, and the need for retransplantation due to HAT.

Choledochal Cyst Excision in Infants-A Retrospective Study.

A choledochal cyst is a rare malformation primarily diagnosed in children. The only effective therapy remains surgical cyst resection followed by Roux-en-Y hepaticojejunostomy. Treating asymptomatic neonates remains a point of discussion. Between 1984 and 2021, we performed choledochal cyst (CC) excision in 256 children at our center. Out of this group, we retrospectively reviewed the medical records of 59 patients who were operated on under one year of age. Follow-up ranged from 0.3 to 18 years (median 3.9 years). The preoperative course was asymptomatic in 22 (38%), while 37 patients (62%) had symptoms before surgery. The late postoperative course was uneventful in 45 patients (76%). In symptomatic patients, 16% had late complications, while in asymptomatic patients, only 4%. Late complications were observed in the laparotomy group in seven patients (17%). We did not observe late complications in the laparoscopy group. Early surgical intervention is not followed by a high risk of complications and may prevent the onset of preoperative complications, giving excellent early and long-term results, especially after minimally invasive laparoscopic surgery.

Case Report: Liver as a Source of Hematopoietic Stem Cells After Liver Transplantation Following Hematopoietic Stem Cell Transplantation.

We report a child with Fanconi anemia who, after hematopoietic stem cell transplantation (HSCT) complicated by acute graft-versus-host disease (GVHD), underwent orthotopic liver transplantation (OLT). Approximately 1 month after OLT, the presence of third-party genetic material from the liver donor was noted and in the next few weeks, the chimerism assessment revealed 100% liver donor leukocytes in the peripheral blood. The rapidly progressing GVHD with gut involvement resulted in patient's death 6 months after OLT. The liver can act as a clinically significant source of hematopoietic stem cells, and the liver donor's young age must be emphasized as potentially predisposing to this phenomenon. Transfer of OLT hematopoietic stem cells may not have clinical significance unless the patient is not immunocompetent or develops liver-transplantation associated GVHD, that can result in lymphocyte mediated elimination of original hematopoiesis. Patients with preexisting immunity disorder (such as primary or secondary immunodeficiency) might require intensified immunosuppressive therapy in peritransplant period as a prevention of liver-transplantation associated GVHD. Close monitoring of hematopoietic chimerism after OLT is warranted in patients at risk, because cytopenia or OLT hematopoiesis can reflect subclinical GVHD and further studies are necessary to elucidate this phenomenon.

Case Report: Sequential Liver After Kidney Transplantation in a Patient With Sensenbrenner Syndrome (Cranioectodermal Dysplasia).

Sensenbrenner syndrome, also known as cranioectodermal dysplasia (CED), is a rare ciliopathy clinically characterized by congenital craniofacial, skeletal, and ectodermal defects. Chronic kidney and liver insufficiency are also present in this disorder. Cranioectodermal dysplasia is an autosomal recessive and heterogeneous genetic disease. Six genes (IFT122, WDR35, IFT140, IFT43, IFT52, and WDR19) are known to be associated with this syndrome. Until 2021 more than 70 patients have been reported with CED, however, an orthotopic liver transplantation has been reported only in one case. Here, we present a case report of sequential liver-after-kidney transplantation in a male patient affected by CED. The kidney and liver transplantation was performed at the age of 7 and 12 years, respectively. Patients with Sensenbrenner syndrome require a multidisciplinary medical management and should regularly be followed-up by hepatologists and nephrologists, as the liver and kidney diseases are the major cause of morbidity and mortality.

Successful Liver Transplantation in Two Polish Brothers with Transaldolase Deficiency.

Transaldolase deficiency (TALDO; OMIM 606003) is a rare inborn autosomal-recessive error of the pentose phosphate pathway. It is an early-onset multisystem disease with dysmorphic features, anaemia, coagulopathy, thrombocytopenia, tubulopathy, hepatosplenomegaly and end-stage liver disease. We present a case of two Polish brothers, born to consanguineous parents, with early-onset TALDO. The dominant feature of disease was an early severe liver injury, with subsequent renal tubulopathy. Nodular liver fibrosis developed in the course of the underlying disease. The older brother presented stable liver function, however, he was qualified for deceased donor liver transplantation (DDLT) because of a liver tumour and suspicion of hepatocarcinoma. The boy was transplanted at the age of 14. The younger brother was qualified for DDLT due to end-stage liver disease and transplanted at the age of 11. Currently, both our patients are alive and in a good condition with normal graft function 23 and 20 months after DDLT respectively. Liver transplantation can be a therapeutic option in TALDO and should be considered in patients with coexisting severe chronic and end-stage liver disease. Long term follow-up is necessary to assess the impact of liver transplantation for quality of life, survival time and the course of the disease.

Achalasia in Children-Clinical Presentation, Diagnosis, Long-Term Treatment Outcomes, and Quality of Life.

BACKGROUND: In spite of the introduction of peroral endoscopic myotomy (POEM), Heller myotomy (HM) remains the mainstay of treatment and the role of pneumatic dilatation (PD) is being debated. The aim of this study was to present a single-center experience in the diagnostic approach and treatment of esophageal achalasia (EA), including the long-term assessment of the QoL. METHODS: Data collection was based on the retrospective analysis of clinical notes and prospective interviews with patients and their parents. RESULTS: The study group consisted of 60 patients with EA (F: 26, M: 34), with a median age of 12.0 (1-17) years at diagnosis. The time from the first symptoms until the diagnosis was 1.0 year (0.5-2.0) and the most common were: regurgitation (91.3%), dysphagia (84.8%), and chest pain (47.8%). The diagnostic approach showed a high sensitivity for barium X-ray follow through, esophageal manometry, and endoscopy. Overall, a long-term good outcome of HM was achieved in 27 out of 37 patients (73%) and it was negatively affected by the time between the first symptoms and the diagnosis. Out of the 16 patients who underwent PD before HM, a good outcome was achieved in 14 patients (87.5%), compared to 13 out of 21 patients (62%) who only underwent HM (p = 0.22). Concomitant fundoplication was routinely performed, and 18% required post-operative endoscopic dilatation. At the end of the 12.1 (0.7-26.6)-year follow up, most patients had a good QoL, which significantly corresponded with the treatment outcomes. CONCLUSIONS: Patients suspected of EA should undergo a thorough clinical evaluation including a manometry, a barium X-ray, and an endoscopy. HM is a safe and effective treatment for achalasia and the outcome is not worsened by a preceding endoscopic PD. In most patients, HM alleviates symptoms, although an impaired QoL is common in long-term follow ups.

Impact of Graft Size Matching on the Early Post-Transplant Complications and Patients Survival in Children after Living Donor Liver Transplantations.

We aimed to assess the impact of the graft-recipient weight ratio (GRWR) on early post-transplant complications and patient survival rates in children after living donor liver transplantation (LDLT). We retrospectively analyzed 321 patients who underwent LDLT from 2004 to 2019. The recipients were categorized into four groups: 37 patients had a GRWR ≤ 1.5% (Group A), 196 patients had a GRWR > 1.5% and ≤3.5% (Group B), 73 patients had a GRWR > 3.5% and <5% (Group C) and 15 patients had a GRWR ≥ 5% (Group D). Incidence of early surgical complications including vascular complications, biliary complications, postoperative bleedings, gastrointestinal perforations and graft loss were comparable among groups with a different GRWR. Delayed abdominal wound closure was more common in patients with a GRWR > 3.5%. Recipients with a GRWR < 5% had a significantly better prognosis concerning patients and graft survival. Using grafts with a GRWR < 5% allows us to expand the donor pool and decrease the risk of mortality while on the waiting list, when patients at the time of transplantation have less advanced liver disease. LDLT with a GRWR ≥ 5% is related to a higher risk of poor outcome, and thus should be an option for treating selected patients when the risk of a delayed transplantation is high and access to deceased donors is limited.

Long-Term Follow-Up of Renal Function in Children after Liver Transplantation-A Single Center Retrospective Study.

Chronic kidney disease (CKD) is a common complication after liver transplantation (LT). Its prevalence with modern immunosuppression regimens, especially in children, is variable depending on the transplantation era. The study included 61 pediatric patients with at least 10 years of follow-up after liver transplantation remaining under constant care of the Department of Pediatric Surgery and Organ Transplantation. The analysis included several tests: estimated glomerular function (eGFR), results of screening for renal tubular defects and blood concentrations of basic immunosuppressive drug-tacrolimus. CKD was diagnosed in 3% of children at 12 years after LT. The maintaining of tacrolimus concentrations >4 ng/mL in long-term observation was associated with a significant increase of microalbuminuria. The presence of microalbuminuria, regarded as a risk factor of CKD, confirmed the necessity of regular comprehensive assessment of patients in long-term follow-up.

Inferior Vena Cava and Venous Outflow Reconstruction in Living Donor Liver Transplantation in Children: A Single-Center Retrospective Study and Literature Review.

BACKGROUND In this report, we present technical problems and solutions used in the reconstruction of the inferior vena cava and graft venous outflow during living donor liver transplantation (LDLT) in children. MATERIAL AND METHODS In 65 grafts out of 379 liver transplantations from living donors, reconstruction of multiple hepatic venous branches and/or IVC was necessary. In 4 cases, cryopreserved deceased donor venous grafts were used for the reconstruction of the IVC and/or HV. RESULTS Follow-up ranged from 2 months to 17.8 years (median 7.2 years). In 4 children, liver re-transplantation was required for a reason not related to venous outflow (biliary complications in 3 patients, graft insufficiency caused by small-for-size syndrome). Two patients died: 1 due to tumor recurrence and 1 due to multi-organ failure. Fifty-nine patients are alive with good liver function. One patient (1.5%) after deceased donor venous graft reconstruction showed symptoms of venous outflow obstruction, which was successfully treated with endovascular balloon angioplasty and stent placement. The remaining 59 transplanted patients do not show any signs of venous outflow obstruction. CONCLUSIONS In most cases, the reconstruction of multiple hepatic veins of living donor allografts can successfully be done with local venoplasty, while using cold-stored vein grafts may be helpful in selected cases of LDLT.

The Course of Ulcerative Colitis After Pediatric Liver Transplantation for Sclerosing Cholangitis.

BACKGROUND: Primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC) are often associated with ulcerative colitis (UC). The impact on the course of UC remains unclear, and up-to-date evidence in pediatric populations is scarce. The aim of the study was to analyze the course of UC in pediatric patients transplanted owing to PSC or ASC. MATERIAL AND METHODS: We retrospectively reviewed data from children with PSC/ASC and UC who underwent orthotopic liver transplantation (OLT). In all patients UC diagnosis was based on clinical presentation, endoscopy, and histology. RESULTS: Seventeen patients (9 female) with PSC or ASC underwent OLT from deceased donors at a median age of 16.8 years (range = 11.5-18.2 years). In 15 patients, UC was diagnosed before OLT (median age of diagnosis = 10.6 years; range = 6.6-18.0 years), and 2 patients developed UC after OLT. Ten patients (59%) presented with pancolitis on initial endoscopy. Disease activity was severe in 9 patients (53%) and most patients improved after initial treatment with steroids. Before OLT only 2 patients (13%) had severe disease activity. After OLT, 4 patients developed flares of the disease. These patients were successfully treated and remained in remission at the end of the posttransplant follow-up period (median = 3.76 years; range = 0.4-15.5 years). None of the patients developed colorectal cancer or underwent colectomy during 3.7 years of post-OLT follow-up. CONCLUSION: In our experience, the course of UC was not aggravated by OLT for PSC, and UC did not adversely affect patient or graft survival.

Health Status in Long-Term Survivors of Hepatoblastoma.

The aim of this study was to evaluate the health status of children cured from hepatoblastoma. Forty-five patients with hepatoblastoma treated between 1996-2014 were assessed. The recorded data included sex, age at diagnosis, disease stage, treatment methods, time since diagnosis, and the evaluation of health status domains which included performance status, growth development, hearing, cardiovascular, skeletal, gastrointestinal, genitourinary, neurological, and hematological function. There were 30 boys and 15 girls. The age at diagnosis ranged from one month to 14 years (median one year). At the time of the health status evaluation, the youngest patient was 5.5 years old and the oldest was 21 years of age (median-10 years). All patients were treated according to the Childhood Liver Tumors Strategy Group-SIOPEL recommendations, though they were not active participants of the studies. The median cumulative dose of cisplatin was 520 mg/m2 and 360 mg/m2 for doxorubicin. Thirty-six patients underwent partial hepatectomy, and nine total hepatectomy and liver transplantation. At a median of nine years from diagnosis, 68% of hepatoblastoma survivors had experienced at least one chronic health condition of any grade. The most frequent late complication was ototoxicity (28.8%), and the most serious were second malignancies (6.6%) and cardiomyopathy (4.4%). Conclusion: Survivors of hepatoblastoma are at risk for long-term complications. They require long-term monitoring for late effects.

Kidney Transplantation in Children with Thrombosed Inferior Caval Vein - Atypical Vascular Anastomoses.

BACKGROUND Diffuse thrombosis of iliac veins and IVC has been considered a significant technical obstacle in pediatric kidney transplantation (KT). MATERIAL AND METHODS Between 1984 and 2018, 951 KTs were performed in our institution. In 4 children qualified for KT, diffuse thrombosis of iliac veins or IVC was found. The surgical techniques, complications, patient and graft survival, and long-term renal function were studied retrospectively. The patients' age at transplantation was 2.5-13 years and body mass was 11-39 kg. RESULTS All children were transplanted with venous anastomoses made to infrahepatic IVC (3 patients) or collateral circulation (1 patient). Early complications developed in 2 patients: significant bleeding from the graft area requiring revision on the second day after transplantation and chyle leak that resolved spontaneously. The follow-up period was 1-12.5 years. Three patients are alive with a follow-up at 7 months, 4.5, and 12 years with serum creatinine 0.7 mg%, 0.6 mg% and 1.4 mg%, respectively. One patient died 1 year after KT, with normal graft function. No late complications related to KT were observed in any patient. CONCLUSIONS Renal transplantation in pediatric patients with thrombotic vascular complications is associated with a number of technical difficulties and problems.

Acute liver failure in children-Is living donor liver transplantation justified?

OBJECTIVES: Living donor liver transplantation (LDLT) in patients with acute liver failure (ALF) has become an acceptable alternative to transplantation from deceased donors (DDLT). The aim of this study was to analyze outcomes of LDLT in pediatric patients with ALF based on our center's experience. MATERIAL AND METHODS: We enrolled 63 children (at our institution) with ALF who underwent liver transplantation between 1997 and 2016. Among them 24 (38%) underwent a LDLT and 39 (62%) received a DDLT. Retrospectively analyzed patient clinical data included: time lapse between qualification for transplantation and transplant surgery, graft characteristics, postoperative complications, long-term results post-transplantation, and living donor morbidity. Overall, we have made a comparison of clinical results between LDLT and DDLT groups. RESULTS: Follow-up periods ranged from 12 to 182 months (median 109 months) for LDLT patients and 12 to 183 months (median 72 months) for DDLT patients. The median waiting time for a transplant was shorter in LDLT group than in DDLT group. There was not a single case of primary non-function (PNF) in the LDLT group and 20 out of 24 patients (83.3%) had good early graft function; 3 patients (12.5%) in the LDLT group died within 2 months of transplantation but there was no late mortality. In comparison, 4 out of 39 patients (10.2%) had PNF in DDLT group while 20 patients (51.2%) had good early graft function; 8 patients (20.5%) died early within 2 months and 2 patients (5.1%) died late after transplantation. The LDLT group had a shorter cold ischemia time (CIT) of 4 hours in comparison to 9.2 hours in the DDLT group (p<0.0001). CONCLUSIONS: LDLT is a lifesaving procedure for pediatric patients with ALF. Our experience showed that it may be performed with very good results, and with very low morbidity and no mortality among living donors when performed by experienced teams following strict procedures.

Acute-on-chronic hepatitis. A case report of autoimmune hepatitis/primary sclerosing cholangitis/ulcerative colitis overlap syndrome in a 15-year-old patient.

Acute-on-chronic liver failure (ACLF) is a disease in which a rapid deterioration of liver function occurs in patients with chronic liver disease, and is usually associated with a precipitating event. We present the case of a boy with autoimmune hepatitis/primary sclerosing cholangitis/ulcerative colitis (AIH/PSC/UC) overlap syndrome, in whom liver function was stable for 4.5 years of treatment. At 15 years of age the patient was hospitalized due to a deterioration of his general condition, severe abdominal pain, diarrhoea, vomiting and weight loss. There was also a rapid deterioration of liver function and a deterioration of renal function. Despite a wide spectrum of diagnostic examinations, no precipitating agent was found. After two episodes of massive bleeding from the gastrointestinal tract, the patient was transferred to the intensive care unit. The patient underwent a successful liver transplantation. ACLF can cause irreversible liver failure with a high mortality rate, which calls for liver transplantation.

Polish Experience with Liver Transplantation and Post-Transplant Outcomes in Children with Urea Cycle Disorders.

BACKGROUND Liver transplantation (LT) is recommended for various metabolic diseases, including urea cycle disorders (UCDs). The aim of this study was to determine indications and outcomes of LT for UCDs in the tertiary reference Children's Hospital in Warsaw, Poland. MATERIAL AND METHODS Medical charts of children with UCD who underwent LT between 2008 and July 2016 were retrospectively reviewed. The following parameters were analyzed: symptoms at time of diagnosis, age at diagnosis, age at transplantation, graft characteristics and survival, postsurgical complications, and biochemical and laboratory results before and after transplantation. RESULTS Twelve patients with UCD who underwent LT at a mean age of 5 y (0.5-14 y) received a total of 14 liver grafts. Four children (33%) received a living donor graft, while 8 (68%) got a deceased donor liver graft. A total number of transplanted organs consisted of 9 (64%) whole-liver grafts and 5 (36%) reduced-size grafts. The 30-day post-transplant patient survival rate was 100% and graft survival rate was 93% (13/14). For those with a post-transplant follow-up of at least 1 year (n=10/12), the 1-year patient survival rate was 100% and the graft survival rate was 85.7% (12/14). Median peak of blood ammonia at presentation was 653 (159-2613) µg/dL (normal <80 µg/dl), and median peak of blood glutamine was 1273.2 µmol/l (964-3900 µmol/l). There was 1 episode of hyperammonemia following LT, but it was not due to UCD. Six (50%) patients were diagnosed with some degree of developmental delay/neurological impairment before transplantation, which remained stable or slightly improved after transplantation. Patients without developmental delay before transplantation maintained their cognitive abilities at follow-up. CONCLUSIONS LT leads to eradication of hyperammonemia, withdrawal of dietary restrictions with low-protein diet, and potentially improved neurocognitive development.

Cavo-portal transposition in pediatric liver transplant recipients.

BACKGROUND: Cavo-portal transposition (CPT) at liver transplantation (LTx) allows portal revascularization of the liver in recipients in whom portal system thrombosis does not allow performance of porto-portal anastomosis. The aim was to present the cases of 2 children who underwent LTx and CPT in our institution. CASE REPORT: 1. A 10-year-old boy, after Kasai procedure and living donor LTx, was qualified for retransplantation 9 years after first LTx complicated with late portal vein thrombosis, portal hypertension, hypersplenism, and multiple GI bleeding episodes, after splenectomy and meso-caval shunt preventing GI bleeding. At retransplant surgery, CPT was done. Actual follow-up was 40 months. Doppler ultrasound and angio CT show normal flow within the graft's portal vein. Biochemical parameters were within normal range. There was no bleeding from the gastrointestinal tract. 2. A 14-month-old child after Kasai procedure was qualified for living donor liver transplantation. During surgery, thrombosis of the recipient portal system was found, which was not diagnosed before. The CPT was done. There were no complications during the postoperative course. The actual follow-up was 32 months, and the patient is doing well, with normal liver and renal function, without hypersplenism or ascites. There was no gastrointestinal bleeding. Doppler ultrasound showed normal intrahepatic portal and arterial flow in the transplanted liver. CONCLUSIONS: Cavo-portal transposition is an important option in portal vein revascularization in liver transplant recipients without access to the portal system. Long-term observation of these 2 cases did not show any late problems (e.g., bleeding from the gastrointestinal tract, renal function, hyperammonemia, ascites) related to cavo-portal transposition.

Non-resectable hepatic tumors in children - role of liver transplantation.

BACKGROUND: There is a group of children with primary hepatic tumors which can not be resected by conventional partial liver resection. Total hepatectomy followed by liver transplantation may be the only solution in such cases. Authors reviewed own experience with the liver transplantation for unresectable tumors in children and assessed the possible indications and role of transplantation in these patients. MATERIAL AND METHODS: Liver transplantation was performed in 17 children with unresectable hepatic tumors out of total number of 350 children transplanted. Hepatocarcinoma was present in 8 children, hepatoblastoma in 6 and benign giant hemangioma in 3. There was no other option for the treatment which would lead to the oncological cure of children with malignant tumors. All patients with giant hemangiomas were infants transplanted urgently due to circulatory and then multiorgan failure. RESULTS: Survival within whole group is 75.5% (13 of 17 pts), 3 children died of malignant tumor recurrence, one of other causes. All 3 children with benign tumors are alive and well. Actual follow-up is from 3 months to 7 years. CONCLUSIONS: Liver transplantation should be considered as option in the treatment of all children with unresectable hepatic tumors. With the careful and individual patient selection significant chances for survival can be achieved in this group of patients which would otherwise not survive with the conventional treatment.