This review aims to synthesize the most updated research, outcomes, and trends in the field of pediatric liver transplantation (LT), specifically focusing on children who have suffered from acute liver failure. Pediatric acute liver failure is a dynamic, life-threatening condition that can either self-resolve or lead to death. LT is a lifesaving intervention. With the introduction of technical variant grafts and recent immunosuppression modifications, overall patient survival, graft survival, and waitlist mortality have improved. Furthermore, recent advances in the knowledge of immunologic mediators of acute liver failure offer the possibility of more detailed understanding of the pathophysiology and new areas for research. Given the success of living donor LT for pediatric patients with acute liver failure, this option should continue to be actively considered as an alternative treatment option for patients who are listed for transplantation and are managed at a multidisciplinary tertiary care transplant center.
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Undifferentiated embryonal sarcoma is the third most common malignant tumor of the liver in children, accounting for 13% of hepatic malignancies in this age group. It has been considered an aggressive neoplasm with very poor prognosis until the late 1980s, when long-term survivors were reported after multiagent chemotherapy followed by resection. We, herein, report two pediatric cases of undifferentiated embryonal sarcoma treated successfully with surgical resection after neoadjuvant chemotherapy based on therapy used in childhood soft tissue sarcomas and in childhood hepatic malignancies. The first patient also had a concurrent cerebellar tumor (pilocytic astrocytoma), for which he first underwent craniotomy and resection, delaying the liver tumor resection by 10 weeks. They are alive and tumor free at 48 months (case no. 1) and 18 months (case no. 2) following neoadjuvant chemotherapy and liver resection.
Management of unresectable pediatric hepatoblastoma (HB) and hepatocellular carcinoma (HCC) remains challenging. The Society of Pediatric Liver Transplantation (SPLIT) database was used to study survival predictors in pediatric liver transplantation (LT) for HB and HCC. Event‐free survival (EFS), associated risk factors, and postoperative complications were studied in children requiring LT for HB/HCC at 16 SPLIT centers. Three‐year EFS was 81% for HB (n = 157) and 62% for HCC (n = 18) transplants. Of HB transplants, 6.9% were PRETEXT II and 15.3% were POST‐TEXT I/II. Tumor extent did not impact survival (p = NS). Salvage (n = 13) and primary HB transplants had similar 3‐year EFS (62% versus 78%, p = NS). Among HCC transplants, 3‐year EFS was poorer in older patients (38% in ≥8‐year‐olds vs 86% <8‐year‐olds) and those with larger tumors (48% for those beyond versus 83% within Milan criteria, p = NS). Risk of infection (HR 1.5, 95% CI 1.1–2.2, p = .02) and renal injury (HR 2.4, 95% CI 1.7–3.3, p < .001) were higher in malignant versus nonmalignant LT. Survival is favorable for pediatric HB and HCC LT, including outcomes after salvage transplant. Unexpected numbers of LTs occurred in PRE/POST‐TEXT I/II tumors. Judicious patient selection is critical to distinguish tumors that are potentially resectable; simultaneously, we must advocate for patients with unresectable malignancies to receive organs.
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