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


METHODS
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.
Baertschiger
Reto Marc
RM
Pediatric Surgery Unit, University of Geneva Children's Hospital, 1211 Geneva 4, Switzerland. reto.m.baertschiger@hcuge.ch
Ozsahin
Hulya
H
Rougemont
Anne-Laure
AL
Anooshiravani
Mehrak
M
Rubbia-Brandt
Laura
L
Le Coultre
Claude
C
Majno
Pietro
P
Wildhaber
Barbara E
BE
Mentha
Gilles
G
Chardot
Christophe
C
D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D006498:Hepatectomy / Q000379:methods*; D018197:Hepatoblastoma / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D013557:Switzerland; D014057:Tomography, X-Ray Computed