Received: 10 March 2017 Revised: 22 August 2017 Accepted: 24 August 2017 DOI: 10.1002/pbc.26820 Pediatric Blood & Cancer The American Society of Pediatric Hematology/Oncology RESEARCH ARTICLE Extreme hepatic resections for the treatment of advanced hepatoblastoma: Are planned close margins an acceptable approach? Adriana Fonseca 1 Abha Gupta 1 Furqan Shaikh 1 Raveena Ramphal 2 Vicky Ng 3 Ian McGilvray 4 J. Ted Gerstle 5 1 Division of Hematology Oncology, The Hospital for Sick Children, University of Toronto, Toronto, Canada 2 Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, Canada 3 Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, Canada 4 Department of Surgery, University Health Network, University of Toronto, Toronto, Canada 5 Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada Correspondence Adriana Fonseca, Division of Hematology Oncology, The Hospital for Sick Children, Univer- sity of Toronto, 555 University Avenue, Toronto, Canada M5G 1 × 8. Email: adriana.fonseca@sickkids.ca Abstract Background: Orthotopic liver transplantation (OLT) is considered the standard for children with hepatoblastoma (HB) in whom complete surgical resection is not possible. However, OLT is not always available or feasible. Objective: To describe the outcome of children with HB who were initially deemed unresectable and underwent complex hepatectomy with planned close margins, and ultimately avoided OLT. Methods: Demographic data, surgical and pathologic details, and survival information were col- lected from children treated for HB between January 2010 to December 2015. Results: Among six children (median age 12 months (3–41 months)), PRETEXT classification was III (n = 2), III/IV (n = 1), and IV (n = 3). Patients received a median of six cycles (range 4–7) of platinum-based induction chemotherapy; five received doxorubicin. Experienced pediatric sur- geons performed extended right and left hepatectomy in five and one patients, respectively, with assistance of an experienced liver transplant surgeon (n = 4). Microscopic margins were positive (n = 2) and negative but close (n = 4; 2–5 mm). Two patients required vascular reconstruction of the vena cava. At median follow-up of 3.3 years (1.7–4.6 years), there was no evidence of local recurrence. One patient had recurrence of pulmonary disease 3 months after surgery. Conclusions: Patients with advanced HB treated with complex surgical resections with positive or close negative margins had good outcomes without OLT. We suggest that planned positive or close microscopic margins in highly selected HB patients may spare the morbidity of OLT and offer an alternative for those ineligible for OLT. Our experience illustrates the importance of a multidisci- plinary team specialized in the management of liver tumors. KEYWORDS hepatoblastoma, outcomes, positive margins, relapse 1 INTRODUCTION Complete surgical resection of the primary tumor is essential to opti- mize good outcomes in children with hepatoblastoma (HB). In several large series of HB, approximately one-third of patients have tumors that can be resected at diagnosis. 1–5 With neoadjuvant chemother- apy, up to 80% of children can achieve complete surgical resec- tion, rendering 20% in need of orthotopic liver transplantation (OLT). Abbreviations: HB, hepatoblastoma; HV, hepatic vein; IVC, inferior vena cava; OLT, orthotopic liver transplantation; PHTT, Pediatric Hepatic Tumour Team; PV, portal vein Historically, incomplete tumor resection and macroscopic residual dis- ease have been associated with poor outcomes. 1,2 Furthermore, cur- rent guidelines recommend OLT in patients that have major vessel involvement: unifocal PRETEXT/POST-TEXT III with tumor ingrowth of all three hepatic veins (HVs) or the retrohepatic vena cava (+V), or portal vein (PV) or both right and left (+P), unifocal PRETEXT/POST- TEXT IV, multifocal PRETEXT/POST-TEXT III and IV. 6–9 There are substantive challenges associated with OLT including limited organ availability, 10,11 risk of relapse especially in patients with synchronous lung metastases, 8,12–14 and the need for life- long immunosuppression. 15 Donor morbidity and mortality also Pediatr Blood Cancer. 2017;e26820. c  2017 Wiley Periodicals, Inc. 1 of 6 wileyonlinelibrary.com/journal/pbc https://doi.org/10.1002/pbc.26820