(1-B) 41. Establish an etiology of PALF in order to identify conditions that are treatable without LT or contraindicated for LT. (1-B) Gold standard treatment of hepatoblastoma (HB) is perioperative chemotherapy followed by complete resection of all viable tumor.[182, 183] The Children’s Oncology Group protocol

for hepatoblastoma (COG-AHEP0731) suggests that tumors with potential for complete resection can be identified after 2-4 rounds of cisplatin-based selleck screening library chemotherapy. Those who undergo primary LT for unresectable HB have an 82% 10-year survival, while those who receive an LT for recurrence of HB following chemotherapy and resection (“rescue” LT) have a 30% 10-year survival.[184] The PRETEXT (Pretreatment Extent of disease)[185] is used to gauge extent of disease at the time of diagnosis and triage patients for early referral to a program with experience in both pediatric hepatobiliary surgery and liver transplantation. Patients with PRETEXT IV disease (disease involving all four sections of liver), complex PRETEXT

III disease (multifocal or presence of venous thrombosis), or centrally located tumors whose location makes a tumor-free excision plane unlikely have poor outcomes with chemotherapy and surgical resection alone.[186] A recent report from a single MCE institution reported 93% survival with aggressive resection MK-8669 in POST-TEXT III and IV patients with hepatoblastoma.[187] Patients with pulmonary metastases (PM) at the time of diagnosis have recurrence-free survival following LT that is similar to those without PM at the time of diagnosis if either of the following occurs following chemotherapy: 1) PM are no longer seen by computerized tomography (CT) or 2) residual PM are completely resected and tumor-free margins are identified.[184] In the absence of significant response to chemotherapy that would

allow surgical resection of the liver tumor with clear margins and sufficient functional residual hepatic mass, total hepatectomy with LT has been demonstrated to have satisfactory long-term outcomes.[188-191] 42. Children with nonmetastatic and otherwise unresectable hepatoblastoma should be referred for LT evaluation at the time of diagnosis or no later than after 2 rounds of chemotherapy. (1-B) 43. Patients with HB and pulmonary metastases can be considered for LT if, following chemotherapy, a chest CT is clear of metastases or, if a tumor is identified, the pulmonary wedge resection reveal the margins are free of the tumor.