Outcomes of children with well-differentiated fetal hepatoblastoma treated with surgery only: Report from Children's Oncology Group Trial, AHEP0731.
J Pediatr Surg
; 57(10): 251-256, 2022 Oct.
Article
em En
| MEDLINE
| ID: mdl-35810020
ABSTRACT
BACKGROUND:
Hepatoblastoma (HB) requires surgical resection for cure, but only 20-30% of patients have resectable disease at diagnosis. Patients who undergo partial hepatectomy at diagnosis have historically received 4-6 cycles of adjuvant chemotherapy; however, those with 100% well-differentiated fetal histology (WDF) have been observed to have excellent outcomes when treated with surgery alone. PATIENTS ANDMETHODS:
Patients on the Children's Oncology Group non randomized, multicenter phase III study, AHEP0731, were stratified based on Evan's stage, tumor histology, and serum alpha-fetoprotein level at diagnosis. Patients were eligible for the very low risk stratum of surgery and observation if they had a complete resection at diagnosis and rapid central histologic review demonstrated HB with 100% WDF histology.RESULTS:
A total of 8 eligible patients were enrolled on study between September 14, 2009 and May 28, 2014. Outcome current to 06/30/2020 was used in this analysis. The median age at enrollment was 22.5 months (range 8-84 months) and the median AFP at enrollment was 714 ng/ml (range 18-77,747 ng/mL). With a median follow-up of 6.6 years (range 3.6-9.8 years), the 5-year event-free (EFS) and overall survival (OS) were both 100%.CONCLUSION:
This report supports that HB with 100% WDF histology completely resected at diagnosis is curable with surgery only. The development of evidence-based surgical guidelines utilizing criteria based on PRETEXT group, vascular involvement (annotation factors), tumor-specific histology and corresponding biology will be crucial for optimizing which patients are candidates for resection at diagnosis followed by observation. LEVEL OF EVIDENCE Prognosis study, Level I evidence.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Hepatoblastoma
/
Neoplasias Hepáticas
Tipo de estudo:
Clinical_trials
/
Guideline
/
Prognostic_studies
Limite:
Child
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Humans
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Infant
Idioma:
En
Revista:
J Pediatr Surg
Ano de publicação:
2022
Tipo de documento:
Article