Management of hepatoblastoma: an update.
OBJECTIVE
To summarize the current standards and guidelines for the diagnosis and management of hepatoblastoma, a rare pediatric liver tumor.


RESULTS
Hepatoblastoma is the most common malignant liver tumor in childhood. International collaborative efforts have led to uniform implementation of the pretreatment extent of disease (PRETEXT) staging system as a means to establish consensus classification and assess upfront resectability. Additionally, current histopathological classification, in light of more advanced molecular profiling and immunohistochemical techniques and integration of tumor biomarkers into risk stratification, is reviewed. Multimodal therapy is composed of chemotherapy and surgical intervention. Achievement of complete surgical resection plays a key role in successful treatment for hepatoblastoma. Overall, outcomes have greatly improved over the past four decades because of advances in chemotherapeutic agents and administration protocols as well as innovations of surgical approach, including the use of vascular exclusion, ultrasonic dissection techniques, and liver transplantation. Challenges remain in management of high-risk patients as well as patients with recurrent or metastatic disease.


CONCLUSIONS
Eventually, a more individualized approach to treating the different types of the heterogeneous spectrum of hepatoblastoma, in terms of different chemotherapeutic protocols and timing as well as type and extent of surgery, may become the basis of successful treatment in the more complex or advanced types of hepatoblastoma.
Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA *Nathalie Kremer and Ashley E. Walther contributed equally to the writing of the article.
Nathalie
N
Kremer
Ashley E
AE
Walther
Gregory M
GM
Tiao
D000971:Antineoplastic Combined Chemotherapy Protocols; D014408:Biomarkers, Tumor; D017024:Chemotherapy, Adjuvant / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018197:Hepatoblastoma / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008113:Liver Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D016031:Liver Transplantation / Q000379:methods*; D009367:Neoplasm Staging; D017410:Practice Guidelines as Topic; D011379:Prognosis; D018570:Risk Assessment; D016896:Treatment Outcome
D014408:Biomarkers, Tumor