Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy.

Pediatric Surgery International
Ryota SouzakiTomoaki Taguchi

Abstract

Pancreatoblastoma (PB) is a rare malignant pancreatic tumor in children and approximately 200 cases have been reported in the literature. The overall 5-year survival rate in PB is 43-50% and no standard treatment for PB has been established. This report presents the case of a 6-year-old female with advanced PB treated successfully by a pylorus-preserving pancreatoduodenectomy (PPPD) after induction chemotherapy, radiation and stem cell transplantation (SCT). A 6-year-old girl was hospitalized for abdominal pain, fever, and vomiting. Abdominal computed tomography (CT) scan showed a 9-cm heterogeneous mass located at the pancreatic head and body, and the duodenum was completely compressed. The inferior vena cava, superior mesenteric artery, and vein were encased by the tumor. The tumor had well-defined margins and calcification. She showed severe anemia and her hemoglobin level was 4.0 g/dl, and the serum alpha-fetoprotein (AFP) level was elevated (884.8 ng/ml). Initially, a resection of the tumor was impossible. An open biopsy was performed and the histopathological diagnosis was PB. She underwent five cycles of the induction chemotherapy regimen for advanced neuroblastoma (cyclophosphamide, etoposide, vincristine, pirarubicin a...Continue Reading

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Citations

Dec 12, 2012·Journal of Pediatric Surgery·Musa IbrahimAipov R Rassulbek
Jun 24, 2011·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·Ewa BienAndrea Ferrari
May 26, 2017·Journal of Pediatric Hematology/oncology·Souji IbukaHiroomi Okuyama
Jun 10, 2015·Journal of Pediatric Hematology/oncology·Chetan Dhamne, Cynthia E Herzog
Jun 27, 2021·Pediatric Blood & Cancer·Ewa BienYves Reguerre
Dec 17, 2020·Journal of Pediatric Hematology/oncology·Giulia ReggianiGianni Bisogno

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