title: Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy.
abstract: BACKGROUND
  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).
  
  
  METHODS
  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 and cisplatin), and the tumor size was decreased to a diameter of 7.5 cm. Furthermore, chemotherapy with irinotecan and vincristine, radiotherapy (40 Gy) and SCT (etoposide, carboplatin, melphalan) was administered. The serum AFP level decreased to 41.1 ng/ml, and the tumor size was decreased to a diameter of 6.5 cm. Then she underwent a PPPD and the tumor was completely resected. The patient's recovery was uneventful, and the AFP returned to the normal values (6.2 ng/ml) after surgery. The child was administered mild postoperative chemotherapy using irinotecan and has been disease-free for 4 months and, and her serum AFP levels remain within normal values.
  
  
  CONCLUSIONS
  This is the first case of PB that was treated with SCT effectively before surgery. The combined therapy including the intensive chemotherapy with SCT and the radiation followed by surgical treatment is thought to be effective for the treatment of advanced PB.
authors.affiliation: Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. ryotas@pedsurg.med.kyushu-u.ac.jp
authors.forename: Ryota
authors.initials: R
authors.lastname: Souzaki
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Tajiri
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Kinoshita
authors.forename: Sakura
authors.initials: S
authors.lastname: Tanaka
authors.forename: Yuhki
authors.initials: Y
authors.lastname: Koga
authors.forename: Aiko
authors.initials: A
authors.lastname: Suminoe
authors.forename: Toshiro
authors.initials: T
authors.lastname: Hara
authors.forename: Kenichi
authors.initials: K
authors.lastname: Kohashi
authors.forename: Yoshinao
authors.initials: Y
authors.lastname: Oda
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Taguchi
mesh_terms: D000970:Antineoplastic Agents / Q000008:administration & dosage* / Q000627:therapeutic use; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D010190:Pancreatic Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D016577:Pancreaticoduodenectomy / Q000379:methods*; D011708:Pylorus / Q000601:surgery*; D018714:Radiotherapy, Adjuvant
chemical_list: D000970:Antineoplastic Agents