Role of Preoperative Hepatobiliary Scintigraphy in Children Requiring Liver Resection.

PURPOSE: The risk of posthepatectomy liver failure (PHLF) remains an important concern following major liver resection. Assessment of future remnant liver function (FRLF) by hepatobiliary scintigraphy has shown its significance to prevent PHLF after major liver resection in adults with a threshold value of FRLF greater than 2.7%/min per m2. However, such data for pediatric patients were not published. METHODS: A total of 77 pediatric patients with liver tumors who underwent 1-stage liver resection were included in this study. Assessment of FRLF, future remnant liver volume (FRLV), and the ratio of remnant liver volume to body weight (RLV-BWR) was performed before the surgery. RESULTS: All patients had RLV-BWR values of more than 0.5%/kg. Future remnant liver volume values ranged from 19% to 89%, and FRLF values ranged from 1.8% to 31.8%/min per m2. Only 7 of 77 patients had FRLV values less than 25%, but their FRLF values exceeded 2.7%/min per m2. Two patients developed grade A and grade B PHLF. CONCLUSION: Future remnant liver volume and the RLV-BWR can be used in most pediatric patients for the assessment of liver before hepatectomy. According to our data, implementation of FRLF assessment using hepatobiliary scintigraphy can be most beneficial for children with FRLV of less than 25%. The cutoff value of FRLV greater than 25% can be slightly decreased with minimal risk of developing PHLF. However, to establish a new cutoff value for FRLV in children, further prospective studies including larger numbers of patients with FRLV of less than 25% are needed.

Case report: First case of undifferentiated embryonal sarcoma of the liver in a child with neurofibromatosis type 1, treated by hepatic chemoperfusion with transcatheter arterial chemoembolization.

Introduction: In this report we firstly describe undifferentiated embryonal sarcoma of the liver (UESL) in a patient with neurofibromatosis type 1 (NF1), fatally complicated by synchronous malignant peripheral nerve sheath tumor (MPNST) with a highly aggressive metastatic course. The case also represents our first experience of chemoperfusion involving the transcatheter arterial chemoembolization (TACE) in a pediatric patient, applied as a treatment for UESL. Case presentation: A 13-year-old girl was diagnosed with NF1 and presented with a liver tumor identified as UESL by histological assessment. The tumor was refractive to the conventional first-line chemotherapy. The patient received hepatic chemoperfusion with TACE, which was efficacious; however, the overall curative outcome was unsatisfactory due to synchronous unresectable retroperitoneal MPNST with mesenteric metastases and ultimate progression of the UESL. Conclusion: This is the first reported case of UESL in a patient with NF1. The results demonstrate the efficacy of hepatic chemoperfusion with TACE in pediatric UESL.

A minimally invasive first stage of ALPPS for hepatoblastoma in a child.

Large and giant tumors, especially hepatoblastomas, are more frequently presented in pediatric patients. At about two-third of hepatoblastomas at the time of diagnosis are unresectable and liver transplantation is considered as a treatment of choice because of insufficient future liver remnant volume. In selected cases, 2-staged hepatectomy may be the unique chance for curative resection except hepatectomy followed by liver replacement. Despite the overwhelming spread of minimally invasive liver surgery, the laparoscopic approach remains uncommon in children. A case of successful partial associating liver partition with portal vein ligation for staged hepatectomy firstly accomplished pure laparoscopically on the first stage in an infant is presented. Described experience suggests both: the feasibility of laparoscopic procedures and the effectiveness of partial in situ liver splitting to reach appropriate future liver remnant volume and to avoid liver transplantation and its disadvantages.