BACKGROUND: “Blastomas” are rare pediatric tumors accounting for less than 1% of tumors in children. First-line treatment consists of chemotherapy and surgical resection. We report, to the best of our knowledge, the first case of a pancreatoblastoma treated by multivisceral transplantation (MVTx).

PATIENT: An 8-year-old boy presented with abdominal tenderness and a palpable mass in the epigastrium. Radiological and histopathological confirmation of pancreatoblastoma were obtained. Chemotherapy was initiated; four cycles of cisplatin and doxorubicin followed by three cycles of cisplatin as single treatment. Due to nephrotoxicity vincristine, actinomycin and cyclophosphamide were given every fourth week for eight cycles while awaiting transplantation. A more than 50% size reduction of the tumor was achieved with this protocol. However, surgical resection was precluded due to tumor involvement of the portal and superior mesenteric veins in addition to multiple jejunal venous branches. MVTx was performed with a graft from an ABO identical donor. Immunosuppression (IS) consisted of induction with anti-thymocyte globulin (ATG), tacrolimus and low-level corticosteroids. Two steroid resistant rejection episodes were managed with additional ATG.  Graft versus host disease (GVHD) with mucocutaneous and hematological manifestations was successfully addressed by steroid administration. Post-transplant lymphoproliferative disorder (PTLD) in the allograft and native colon was documented with a concomitant rise in Epstein-Barr virus (EBV) titers. This was resolved after three courses of rituximab followed by four courses of mCOMP (cyclophosphamide, vincristine, methotrexate, prednisolone). The patient was discharged with partial parenteral nutrition (PN) after 10 months. Radiological follow-up as well as alfa-fetoprotein levels remain normal 18 months after transplantation. CONCLUSION: MVTx may be an option in selected cases of non-resectable intra-abdominal malignancies. The post-transplant course in this case was strongly influenced by the additional toxicity and immunosuppressive properties of the pre- and post-transplant chemotherapy. Long term follow-up is still lacking.