Multivisceral Transplantation for Unresectable Hepatoblastoma With Mesenteric Venous Invasion or Non-Adapting Short Bowel Syndrome: A Case Series

Background: Liver transplantation is the standard of care for unresectable hepatoblastoma.In rare situations, isolated liver transplantation is insufficient to achieve complete clearance of the tumor.We report 3 pediatric patients who underwent multivisceral transplantation (liver-small bowelpancreas composite) with contraindications to isolated liver transplantation.Methods: Medical records of those patients with hepatoblastoma who underwent multivisceral transplant at a large pediatric transplant center were reviewed.All were treated with a minimum of 5 cycles of neoadjuvant chemotherapy (Patient A: Cisplatin x 5 cycles; Patient B: Cisplatin/5-Florouracil/Vincristine/Doxorubicin x 6 cycles, Vincristine/ Irinotecan x 8 cycles, Cisplatin x 1, Patient C: Cisplatin/ Doxorubicin x 3 cycles, Irinotecan x 1 cycle, Ifosfamide/ Carboplatin/Etoposide x 1 cycle).Induction immunosuppression included anti-thymocyte globulin and steroids.Results: Three patients (age <3 years) underwent liverpancreas-small bowel transplant for unresectable hepatoblastoma.Patients A and B had POSTTEXT IV disease with diffuse mesenteric tumor thrombus.Patient C had a history of short bowel syndrome due to necrotizing enterocolitis and severe intestinal failure-associated liver disease with POSTTEXT IV hepatoblastoma.One patient's single episode of acute rejection in the first year, responded to steroids and antithymocyte globulin.None of the three experienced oncologic recurrences over a mean follow-up of 3 years.Two patients are alive with excellent graft health, one patient succumbed to severe graft-versus-host disease 2 years following transplant.Conclusions: Multivisceral transplantation can be performed in selected cases of unresectable hepatoblastoma, not amenable to liver transplant alone.Our initial experience with these 3 patients demonstrates an acceptable post-transplant disease-free survival.