Embryonal sarcoma of the liver.

PURPOSE: Embryonal sarcoma accounts for 6 % of liver tumors. This study reviews its features and the results of treatment in a referral center. METHODS: We retrospectively reviewed liver tumors treated between 1995 and 2011. PRETEXT staging and biopsy were used to tailor chemotherapy according to SIOP protocols. Radical surgery was performed when possible. Complications and cumulative survival were the outcome endpoints. RESULTS: Six out of 156 primary liver tumors (four males and two females) were sarcomas. The mean age at diagnosis was 81 ± 8.5 months. The most frequent finding was abdominal mass. Alfa-fetoprotein was normal. Imaging depicted heterogeneous tumors with septa, necrosis, and hemorrhagic areas. The diagnosis was ascertained by biopsy. Three tumors were located in the right lobe (PRETEXT II): two were bilobar (PRETEXT III) and one was in the left lobe (PRETEXT I). Two children had metastases at diagnosis and high-risk chemotherapy (vincristine, carboplatin, epirubicin) was administered with poor response. They died without operation 4 and 10 months later. Four patients with local disease underwent typical liver resections after chemotherapy (iphosphamide, vincristine, actinomycin D, and doxorrubicin). Overall actuarial survival at 70 months was 66.6 %. CONCLUSIONS: Extended and metastatic embryonal sarcoma do poorly whereas localized tumors amenable to complete surgical removal after chemotherapy can cure.

[Liver transplant from living donor]. / Trasplante hepático de donante vivo.

AIM: Even though Spain has the highest donation rate in the world, our needs cannot be satisfied, specially in younger children. Living-related donor transplant is an alternative in those cases. PATIENTS AND METHOD: We performed a retrospective study of 57 living-related donor transplants performed in our hospital between June 1993 and December 2007. Median age and weight were 1.2 years old (0.5-14.8) and 8.5 kg (5-62). Indications for transplant were as follow: biliary atresia in 42 cases (73.7%), hepatic tumor in 8 (14%) and others in 7 patients. Type of graft was: monosegment (n=1), left lateral segment (n=45), extended left lateral segment (n=5), left liver (n=4), right liver (n=2). We studied the following factors: graft and patient survival (Kaplan Meier), perioperative conditions, complications, causes of graft lost, donor complications and technique difficulties. RESULTS: Patient survival at 3 months, 1 year, 5 years and 10 years was 98.2%, 98.2%, 95% and 95% respectively. Three grafts werelost due to arterial thrombosis, two due to rejection, one due to portal thrombosis and three due to other causes. Complications were as follow: biliary fistula in the cut surface (6), biliary anastomosis complications (6), cut surface abcess (1), portal stenosis (2), suprahepatic stenosis (1) and intestinal perforation (2). Most common complication in donors was biliary leak (4). Among the technique difficulties, 8 patients needed major reconstruction of suprahepatic vein; 4 needed complex portal reconstruction, 6 patients had double biliary tract and 4 patients needed multiple arterial anastomosis. Wall closure was delayed (Goretex) in 35% of cases (20). CONCLUSIONS: Despite technical complications, results after living-related donor transplantation are excellent. It is particularly favourable for children with low weight, since Spanish policy for organ allocation does not make easy to find an adecuate donor in short periods of time. Without living-related donor transplantations, mortality pretransplant would be much higher.

[Liver bipartition as an alternative to the transplant]. / Bipartición hepática como alternativa en el trasplante.

AIM: Liver pediatric transplantation finds in the lack of donors its main limitation. An alternative in those cases is split liver grafts from bigger donors. PATIENTS AND METHOD: We performed a retrospective study of 56 hepatic split transplants performed between 1994 and 2007. Twenty-nine children were transplanted with a median age and weight of 1.8 years old (0.3-9) and 9.7 kg (6.2-23). In 16 cases (53.3%) liver transplant was performed in emergency situation. In one patient we performed a combined transplant (liver-kidney) and in another patient it was a second transplant due to primary graft failure after receiving an hepatointestinal allograft. Type of grafts used were: lateral left segment (n=26), extended lateral left segment (n=1) and extended right liver (n=3). Median donor age and weight were 20 years old (8-44) and 60 kg (24-80). We studied patient and graft survival (Kaplan Meier), perioperative factors, complications and net rate of early complications in adults recipients. RESULTS: Patient survival was 96.7% after 6 months, 1 year, 5 years and 10 years. Id for grafs 86.7%. Two grafts were lost due to arterial thrombosis, one due to primary non function and another due to recipient death secondary to a sepsis. Five children had major biliary complications and 2 of them developed multiple intrahepatic stenoses, one of them being on waiting list for retransplant. Early graft lost (retransplant or death before leaving the hospital) occurred in 4 out of the 25 grafts transplanted in other centers (25 adults, 1 kid); all of them occurred in the initial period (1994-2001). CONCLUSIONS: Even though it is clearly documented that benefit of transplant (measured in years of life won) is very good after split transplantation, nowadays criteria for organ allocation in Spain do not allow a more extensive diffusion of this technique and it is confined to urgent transplant. Even in those cases, results after split transplantation are excellent. Without this possibility our pretransplant mortality would be much higher.

Comparative study between living and cadaveric donors in pediatric liver transplantation.

UNLABELLED: We examined whether the results in living-related hepatic transplantation (LRLT) are better than those from a cadaveric donor (CDLT). MATERIAL AND METHODS: The last 27 consecutive LRLT, performed from 1998 to 2005, were compared with 27 CDLT matched for age, weight, date, and diagnosis. Grafts in LRLT group were left lateral segment (n = 22), left lobe (n = 3), and right lobe (n = 2). In the CDLT group, the grafts were split in situ (n = 10), hepatic reduction (n = 9) and whole liver (n = 8). We analyzed the actuarial survivals (grafts and children), retransplantation, primary nonfunction, initial graft malfunction (liver enzymes >2000 U/L), surgical complications, rejection, and resource consumption. RESULTS: Patient survivals at 6 months, 1 year, and 5 years were 100%, 96%, and 96% in LRLT and 100%, 100%, and 100% in CDLT (P = NS). Graft survivals were 93%, 89%, and 89% versus 96%, 96%, and 96%, respectively (P = NS). Complications were biliary complications (LRLT, 25% vs CDLT, 3%; P = .021); portal vein thrombosis (LRLT, 7% vs CDLT, 3%; NS), and hepatic artery thrombosis (LRLT, 0% vs CDLT, 3%; NS). The overall incidence of acute rejection was slightly higher (NS) in LRLT (LRLT, 18% vs CDLT, 11%; NS). Liver enzyme levels were higher in the CDLT group, but initial malfunction rate was not statistically different. Regarding resource consumption: blood product needs were higher in LRLT (P < .05) and hospital stay and ICU stay were longer, although not significantly, among LRLT. CONCLUSIONS: The results in LRLT among children are similar to those obtained in CDLT. We found a trend towards less initial graft malfunction in LRLT. Blood product needs were higher in LRLT. Hospital and ICU stay were longer, but not significantly different in LRLT. The benefits of LRLT are saving a scarce resource: a cadaveric donor liver graft.