Morgan E R, Perryman J H, Reynolds M, Gonzalez-Crussi F J
Department of Pediatrics, Children's Memorial Hospital, Chicago, Illinois, USA.
Med Pediatr Oncol. 1996 Apr;26(4):284-92. doi: 10.1002/(SICI)1096-911X(199604)26:4<284::AID-MPO13>3.0.CO;2-F.
A case is reported of a 7-year old girl diagnosed with initially inoperable metastatic embryonic pancreatic tumor, which showed a significant clinical response to chemotherapeutic agents commonly used to treat hepatoblastoma. This regimen was selected because certain histologic features of the tumor demonstrated characteristics seen in hepatic tissue. After two course of chemotherapy (cis-platinum and adriamycin), there was a significant reduction of the primary mass, and it was completely resected, although the tumor subsequently recurred in the metastatic, unoperated site. The embryological relationship between the tumor in this patient and hepatoblastoma, as well as the literature concerning treatment in pediatric pancreatoblastic tumors are reviewed. Complete eradication of tumor appears to be necessary for cure.