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Cure of multifocal panhepatic hepatoblastoma: is liver transplantation always necessary?

Anooshiravani, Mehrak
Le Coultre, Claude
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Published in Journal of pediatric surgery. 2010, vol. 45, no. 5, p. 1030-1036
Abstract PURPOSE: Multifocal panhepatic hepatoblastoma (HB) without extrahepatic disease is generally considered as an indication for total hepatectomy and liver transplantation. However, after initial chemotherapy, downstaging of the tumor sometimes allows complete macroscopic resection by partial hepatectomy. This procedure is no longer recommended because of the risk of persistent viable tumor cells in the hepatic remnant. We report our experience with conservative surgery in such cases. METHOD: Between 2000 and 2005, 4 children were consecutively referred to our unit with multinodular pan-hepatic HBs (classification PRETEXT IV of the International Society of Pediatric Oncology Liver Tumor Study Group SIOPEL). Three of them had extrahepatic disease at diagnosis. All patients were treated according to SIOPEL 3 and 4 protocols. RESULTS: Extrahepatic metastases were still viable in 2 of 3 patients after initial chemotherapy. These patients eventually died of tumor recurrence. In the 2 patients without residual extrahepatic disease, liver tumors had regressed, and complete macroscopic excision of hepatic tumor remnants could be achieved by conservative surgery. These 2 children are alive and well and free of tumor 7 years after diagnosis. CONCLUSIONS: Conservative surgery may be curative in some multinodular PRETEXT IV HB patients, with a good response to preoperative chemotherapy and complete excision of all macroscopic tumor remnants. However, because of the lack of reliable predictors of sterilization of the microscopic disease in the residual liver, with subsequent poor prognosis, total hepatectomy and liver transplantation remain currently recommended in patients with multinodular PRETEXT IV HB without extrahepatic disease, even though some of these children are probably overtreated.
Keywords Chemotherapy, AdjuvantChild, PreschoolHepatectomy/ methodsHepatoblastoma/drug therapy/pathology/ surgeryHumansInfantLiver Neoplasms/drug therapy/pathology/ surgeryLiver TransplantationMaleSwitzerlandTomography, X-Ray Computed
PMID: 20438949
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Other version: http://www.sciencedirect.com/science/journal/00223468
Research groups Groupe Ozsahin Ayse Hulya (onco-hémato pédiatrie) (889)
Hépatologie chirurgicale (327)
Métastases du foie (657)
Recherche clinique en chirurgie pédiatrique (886)
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BAERTSCHIGER, Reto Marc et al. Cure of multifocal panhepatic hepatoblastoma: is liver transplantation always necessary?. In: Journal of pediatric surgery, 2010, vol. 45, n° 5, p. 1030-1036. doi: 10.1016/j.jpedsurg.2010.01.038 https://archive-ouverte.unige.ch/unige:20754

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Deposited on : 2012-05-23

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