Liver transplantation in the management of unresectable hepatoblastoma in children
University of Utah, Primary Children's Medical Center, Salt Lake City, UT 84113, USA.Frontiers in bioscience (Elite edition) 01/2012; 4(4):1293-302.
Complete surgical resection is essential to long-term survival in children with hepatoblastoma. We present the guidelines from the Children's Oncology Group (COG), liver tumor study group of the Societe Internationale Oncologie Pediatrique (SIOPEL), and German Pediatric Oncology Group (GPOH) for early referral of children with potentially unresectable hepatoblastoma to a specialty center with expertise in extreme resection and liver transplantation. Patients who will become candidates for liver transplantation should receive chemotherapy following the same protocols as for children undergoing a partial hepatectomy. The Pediatric Liver Unresectable Tumor Observatory (PLUTO) is an international prospective database established to collect data and make future recommendations on controversial issues regarding the use of transplant in hepatoblastoma including: 1) What is the optimal treatment of multifocal tumors. 2) What is the role of extreme resection vs. liver transplant in patients with major venous involvement. 3) What is the role of transplant in patients who present with lung metastasis. 3) Should patients with tumor relapse be offered a rescue transplant. 4) What is the role of pre- and post- transplant chemotherapy.
Article: Surgical treatment of hepatoblastoma[Show abstract] [Hide abstract]
ABSTRACT: Surgical resection remains the cornerstone of cure in hepatoblastoma (HB). Meticulous review of contrast enhanced CT/MR imaging facilitates PRETEXT and POST-TEXT grouping to determine optimal timing and desired extent of liver resection. Excellent knowledge of liver anatomy is essential and the dissection must ensure protection of the vascular inflow and outflow to the remaining liver at all times. Referral to a liver specialty center in advanced cases may facilitate resectability. Potential surgical complications include bleeding, vascular injury, cardiac arrest, liver failure, and bile leak. The risk of complications can be minimized with preoperative planning, appropriate referral, and precise surgical technique. Pediatr Blood Cancer 2012; 59: 800-808. © 2012 Wiley Periodicals, Inc.
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ABSTRACT: Purpose of review: This review outlines the current approaches to prenatal imaging, differential diagnosis, antenatal natural history, and the available treatment options for the most commonly prenatally diagnosed malignant tumors. Recent findings: In-utero diagnosis of fetal tumors, although still a rare event, has become more common as prenatal imaging modalities have improved. In general, this prenatal diagnosis allows more informed prenatal counseling and better perinatal planning for potentially high-risk deliveries. There are rare indications for prenatal fetal intervention. Summary: Diagnosis of a fetal tumor should prompt referral to a specialized center. Further understanding of these rare patients will require multicenter collaboration.
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ABSTRACT: Background: Liver transplantation (LTx) for hepatoblastoma appears to be increasing. Favorable tumor histology is increasingly linked to survival after surgical resection and could also determine posttransplantation outcomes. Methods: To evaluate national trends in tumor and LTx incidence as the basis for observations at some LTx centers, and determinants of survival after LTx for hepatoblastoma, we queried the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) registry representing 9.451% of the U.S. population (1975-2007), the United Network for Organ Sharing (UNOS, 1988-2010, n = 332), and Children's Hospital of Pittsburgh database (CHP, 1987-2011, n = 35). Results: In the United States, hepatoblastoma cases increased 4-fold, LTx for hepatoblastoma increased 20-fold, and hepatoblastoma surpassed other unresectable liver malignancies requiring LTx by nearly 3-fold. Actuarial 5-year patient survival exceeded 75%. Recurrences in 16% were greater after segmental LTx in the total U.S. experience (P = .049). At CHP, 5 children died from recurrences (n = 4) and sepsis (n = 1). Tumors were epithelial (57%) or mixed epithelial-stromal (42%), Children's Oncology Group stage III (77%) or IV (23%). Recurrences were related to previous pulmonary metastases (P = .016), and tumor necrosis <50% (P = .013), but not to small cell undifferentiated tumor histology (P = NS). Hepatic artery thrombosis was more common after LTx for hepatoblastoma compared with nonmalignant indications (P = .0089). Thirty-three children received pre-LTx chemotherapy, 88.6% with cisplatin, and 85.7% received post-LTx chemotherapy. Conclusion: Outcomes after LTx for hepatoblastoma may benefit from improved detection and treatment of pretransplantation metastases, adequate tumor lysis after chemotherapy, and perioperative antithrombotic agents but are unaffected by undifferentiated tumor histology.