Changing treatment and outcome of children with hepatoblastoma: analysis of a single center experience over the last 20 years.
OBJECTIVE
The aim of the study was to analyze changing management and survival of children with hepatoblastoma (HBL) treated in one center.


METHODS
Over the last 20 years, 51 children with HBL were treated. Surgery was performed in 48 children (94.1%), conventional liver resection in 38 (of those, 2 received a rescue liver transplantation [LTx] for relapse), and total hepatectomy and primary LTx in 10 patients. The remaining 3 patients received only palliative treatment. Patient data were analyzed for survival with respect to PRETreatment EXTent of disease (PRETEXT), metastases, histopathology, conventional resection, and LTx.


RESULTS
Survival of children with HBL treated with liver resection is 71% and 80% for primary LTx. Favorable prognostic factors for patient survival was tumor histology as epithelial-fetal subtype and mixed epithelial and mesenchymal type, without teratoid features, and good response to chemotherapy (necrosis, fibrosis). Unfavorable prognostic factors were small cells undifferentiated, transitional liver cell tumor, α-fetoprotein level above 1,000,000 IU/mL and below 100 IU/mL at diagnosis, lung metastases, and local recurrence after initial resection. Survival was related to PRETEXT stage. However, among patients with PRETEXT III and IV, LTx resulted in better survival.


CONCLUSIONS
Liver transplantation is a good option for children with advanced HBL. Early referral of children with potentially unresectable tumors to centers where combined treatment (chemotherapy, surgery including LTx) is available is crucial.
Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, 04-730 Warsaw, Poland. h.ismail@czd.pl
Hor
H
Ismail
Dorota
D
Broniszczak
Piotr
P
Kaliciński
Bożenna
B
Dembowska-Bagińska
Danuta
D
Perek
Joanna
J
Teisseyre
Przemyslaw
P
Kluge
Andrzej
A
Kościesza
Agnieszka
A
Lembas
Malgorzata
M
Markiewicz
D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy / Q000379:methods / Q000639:trends*; D018197:Hepatoblastoma / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods / Q000639:trends*; D008297:Male; D020360:Neoadjuvant Therapy; D010166:Palliative Care; D016019:Survival Analysis; D016896:Treatment Outcome
D000970:Antineoplastic Agents; D004317:Doxorubicin; D002945:Cisplatin