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.

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.

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.