https://www.ncbi.nlm.nih.gov/labs/articles/27501172/

Fuchs J1, Cavdar S, Blumenstock G, Ebinger M, Schäfer JF, Sipos B, Warmann SW. Ann Surg. 2017 Aug;266(2):318-323. doi: 10.1097/SLA.0000000000001936.

Abstract

OBJECTIVE:
To analyze the outcome of hepatoblastoma (HB) patients presenting with post treatment extent of disease (POST-TEXT) stages III and IV after neoadjuvant chemotherapy.

BACKGROUND:
Primary liver transplantation has been advocated as surgical treatment for children with HB involving 3 or 4 sectors at diagnosis. However, in some cases, tumors seem resectable after chemotherapy through aggressive use of nontransplant surgical procedures.

METHODS:
Data of 27 HB patients were reviewed, undergoing extended liver resection for POST-TEXT III or IV tumors after chemotherapy between 1992 and 2015. Median follow-up was 58 months (range 9-188).

RESULTS:
Median age at surgery was 18.2 months (interquartile range 10.8-32.5). Staging of the children after chemotherapy revealed POST-TEXT III in 21 and POST-TEXT IV in 6 cases. In 2 children, the hepatic resection was performed under cardiopulmonary bypass because of extended vena cava thrombosis; in 2 patients, a simultaneous sternotomy was performed for resection of bilateral lung metastases. The 5-year overall survival rate was 80.7%.

CONCLUSIONS:
Aggressive surgical resection is a successful approach in some patients with POST-TEXT III and IV HB who otherwise would be candidates for liver transplantation. These children should undergo central review and should be surgically managed at centers of excellence for pediatric liver surgery. Despite challenging surgical procedures and complex clinical courses, the patients benefit from avoidance of morbidities of organ transplant. However, preparation of backup liver transplantation should be considered in selected cases.