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

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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).

Case report

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.

Conclusion

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.

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References

  1. Dall’igna P, Cecchetto G, Bisogno G, Conte M, Chiesa PL, D’Angelo P, De Leonardis F, De Salvo G, Favini F, Ferrari A, on Behalf of the TREP Group (2009) Pancreatic tumors in children and adolescents: The Italian TREP Project Experience. Pediatr Blood Cancer doi:10.1002/pbc

  2. Shorter NA, Glick RD, Klimstra DS, Brennan MF, Laquaglia MP (2002) Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present. J Pediatr Surg 37(6):887–892

    Article  PubMed  Google Scholar 

  3. Dhebri AR, Connor S, Campbell F, Ghaneh P, Sutton R, Neoptolemos JP (2004) Diagnosis, treatment and outcome of pancreatoblastoma. Pancreatology 4:441–453

    Article  CAS  PubMed  Google Scholar 

  4. Savastano S, d’Amore ES, Zuccarotto D, Banzato O, Beghetto M, Famengo B (2009) Pancreatoblastoma in an adult patient. A case report. JOP 10(2):192–195

    Google Scholar 

  5. Levey JM, Banner BF (1996) Adult pancreatoblastoma: a case report and review of the literature. Am J Gastroenterol 91(9):1841–1844

    CAS  PubMed  Google Scholar 

  6. Horie A, Yano Y, Kotoo Y, Miwa A (1977) Morphogenesis of pancreatoblastoma, infantile carcinoma of pancreas. Report of two cases. Cancer 39:247–254

    Article  CAS  PubMed  Google Scholar 

  7. Yonekura T, Kosumi T, Hokim M, Hirooka S, Kitayama H, Kubota A (2006) Aggressive surgical and chemotherapeutic treatment of advanced pancreatoblastoma associated with tumor thrombus in portal vein. J Pediatr Surg 41:596–598

    Article  PubMed  Google Scholar 

  8. Ghavamzadeh A, Alimoghaddam K, Jahani M, Mousavi SA, Iravani M, Bahar B, Khodabandeh A, Khatami F, Gaffari F, Jalali A (2009) Stem cell transplantation; Iranian experience. Arch Iran Med 12(1):69–72

    PubMed  Google Scholar 

  9. Murakami T, Ueki K, Kawakami H, Gondo T, Kuga T, Esato K, Furukawa S (1996) Pancreatoblastoma: case report and review of treatment in the literature. Med Pediatr Oncol 27(3):193–197

    Article  CAS  PubMed  Google Scholar 

  10. Défachelles AS, Martin De Lassalle E, Boutard P, Nelken B, Schneider P, Patte C (2001) Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients. Med Pediatr Oncol 37(1):47–52

    Article  PubMed  Google Scholar 

  11. Muguerza R, Rodriguez A, Formigo E, Montero M, Vázquez JL, Páramo C, Campos C (2005) Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature. J Pediatr Surg 40(8):1341–1344

    Article  PubMed  Google Scholar 

  12. Willnow U, Willberg B, Schwamborn D, Körholz D, Göbel U (1996) Pancreatoblastoma in children. Case report and review of the literature. Eur J Pediatr Surg 6(6):369–372

    Article  CAS  PubMed  Google Scholar 

  13. Pappo AS, Lyden E, Breitfeld P, Donaldson SS, Wiener E, Parham D, Crews KR, Houghton P, Meyer WH; Children’s Oncology Group (2007) Two consecutive phase II window trials of irinotecan alone or in combination with vincristine for the treatment of metastatic rhabdomyosarcoma: the Children’s Oncology Group. J Clin Oncol 25(4):362–369

    Google Scholar 

  14. Inagaki J, Yasui M, Sakata N, Inoue M, Yagi K, Kawa K (2005) Successful treatment of chemoresistant stage 3 neuroblastoma using irinotecan as a single agent. J Pediatr Hematol Oncol 27(11):604–606

    Article  PubMed  Google Scholar 

  15. Tsirlis T, Vasiliades G, Koliopanos A, Kopanakis N, Katseli A, Tsipras H, Margaris H (2009) Pancreatic leak related hemorrhage following pancreaticoduodenectomy. A case series. JOP 10(5):492–495

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Acknowledgments

We thank Dr. Ryosuke Miyaji (Department of Pediatrics and Transfusion Medicine, School of Medicine, University of Occupational and Environmental Health, Japan) and Masanori Hisaoka (Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, Japan) for the contributions to this case.

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Correspondence to Ryota Souzaki.

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Souzaki, R., Tajiri, T., Kinoshita, Y. et al. Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy. Pediatr Surg Int 26, 1045–1048 (2010). https://doi.org/10.1007/s00383-010-2655-9

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  • DOI: https://doi.org/10.1007/s00383-010-2655-9

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