Artigo Revisado por pares

Changing treatment and outcome of children with hepatoblastoma: analysis of a single center experience over the last 20 years

2012; Elsevier BV; Volume: 47; Issue: 7 Linguagem: Inglês

10.1016/j.jpedsurg.2011.11.073

ISSN

1531-5037

Autores

Hor Ismail, Dorota Broniszczak, Piotr Kaliciński, Bożenna Dembowska–Bagińska, Danuta Perek, Joanna Teisseyre, Przemysław Kluge, Andrzej Kościesza, Agnieszka Lembas, M Markiewicz,

Tópico(s)

Organ Transplantation Techniques and Outcomes

Resumo

The aim of the study was to analyze changing management and survival of children with hepatoblastoma (HBL) treated in one center.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.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.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.

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