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Pancreatic Neoplasms in Children

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The Surgery of Childhood Tumors

Abstract

Pancreatic neoplasms in children are very uncommon, representing less than 1 % of all solid tumors. Because of their rarity, our understanding of their natural history is limited. Pancreatic neoplasms are divided into epithelial and non-epithelial categories. Epithelial tumors are those with a cell line that resembles the lining of the pancreatic ducts, the lining of the pancreatic acini or the cells that form the islets of Langerhans, which can be functional or clinically silent. Non-epithelial tumors are those that arise from tissue of mesenchymal or ectodermal origin, which are extremely rare. In general, pancreatic neoplasms in children have an overall better prognosis than in adults, although some tumors in children are very aggressive, unresectable at diagnosis and have a poor survival rate. Among all different types of pancreatic neoplasms in children, pancreatoblastoma (PBT) and solid-pseudopapillary tumor (SPPT) are the most common ones in the first and second decade of life, respectively. Complete surgical resection is the key in the treatment of all pancreatic neoplasms in children.

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References

  1. Surveillance, Epidemiology and End Results (SEER) Program, National Cancer Institute, National Institutes of Health, Bethesda. www.seer.cancer.gov .

  2. Classification of tumors of the digestive system, 4th ed. Lyon: International Agency for Research on Cancer, World Health Organization: 2010.

    Google Scholar 

  3. Cavallini A, Falconi M, Bortesi L, et al. Pancreatoblastoma in adults: a review of the literature. Pancreatology. 2009;9(1–2):73–80.

    Article  PubMed  Google Scholar 

  4. Kerr NJ, Chun YH, Yun K, et al. Pancreatoblastoma is associated with chromosome 11p loss of heterozygosity and IGF2 overexpression. Med Pediatr Oncol. 2002;39(1):52–4.

    Article  PubMed  Google Scholar 

  5. Suchi M, MacMullen CM, Thornton PS, et al. Molecular and immunohistochemical analyses of the focal form of congenital hyperinsulinism. Mod Pathol. 2006;19(1):122–9.

    Article  CAS  PubMed  Google Scholar 

  6. Kletter GB, Sweetser DA, Wallace SF, et al. Adrenocorticotropin-secreting pancreatoblastoma. J Pediatr Endocrinol Metab. 2007;20(5):639–42.

    PubMed  Google Scholar 

  7. Chisholm KM, Hsu CH, Kim MJ, et al. Congenital pancreatoblastoma: report of an atypical case and review of the literature. J Pediatr Hematol Oncol. 2012;34(4):310–5.

    Article  PubMed  Google Scholar 

  8. Bien E, Godzinski J, Dall’igna P, et al. Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). Eur J Cancer. 2011;47(15):2347–52.

    Article  PubMed  Google Scholar 

  9. Défachelles AS, Martin De Lassalle E, Boutard P, et al. Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients. Med Pediatr Oncol. 2000;37(1):47–52.

    Article  Google Scholar 

  10. Frantz VK. Tumors of the pancreas. Atlas of tumor pathology VII, fascicles 27 and 28. Washington, DC: Armed Forces Institute of Pathology; 1959. p. 32–3.

    Google Scholar 

  11. Guo Y, Yuan F, Deng H, et al. Paranuclear dot-like immunostaining for CD99: a unique staining pattern for diagnosing solid-pseudopapillary neoplasm of the pancreas. Am J Surg Pathol. 2011;35(6):799–806.

    Article  PubMed  Google Scholar 

  12. Cheuk W, Beavon I, Chui DT, et al. Extrapancreatic solid pseudopapillary neoplasm: report of a case of primary ovarian origin and review of the literature. Int J Gynecol Pathol. 2011;30(6):539–43.

    Article  PubMed  Google Scholar 

  13. van den Akker M, Angelini P, Taylor G, et al. Malignant pancreatic tumors in children: a single-institution series. J Pediatr Surg. 2012;47(4):681–7.

    Article  PubMed  Google Scholar 

  14. Tipton SG, Smyrk TC, Sarr MG, et al. Malignant potential of solid pseudopapillary neoplasm of the pancreas. Br J Surg. 2006;93:733e7.

    Article  Google Scholar 

  15. Goh BK, Tan YM, Cheow PC, et al. Solid pseudopapillary neoplasms of the pancreas: an updated experience. J Surg Oncol. 2007;95(8):640–4.

    Article  PubMed  Google Scholar 

  16. Butte JM, Brennan MF, Gönen M, et al. Solid pseudopapillary tumors of the pancreas. Clinical features, surgical outcomes, and long-term survival in 45 consecutive patients from a single center. J Gastrointest Surg. 2011;15(2):350–7.

    Article  PubMed  Google Scholar 

  17. Gomez P, Yorke R, Ayala AG, et al. Solid-pseudopapillary neoplasm of pancreas with long delayed liver metastasis. Ann Diagn Pathol. 2011;16(5):380–4.

    Article  PubMed  Google Scholar 

  18. Hah JO, Park WK, Lee NH, et al. Preoperative chemotherapy and intraoperative radiofrequency ablation for unresectable solid pseudopapillary tumor of the pancreas. J Pediatr Hematol Oncol. 2007;29(12):851–3.

    Article  PubMed  Google Scholar 

  19. Kanter J, Wilson DB, Strasberg S. Downsizing to resectability of a large solid and cystic papillary tumor of the pancreas by single-agent chemotherapy. J Pediatr Surg. 2009;44(10):e23–5.

    Article  PubMed  Google Scholar 

  20. Zamboni G, Terris B, Scarpa A, et al. Acinar cell cystadenoma of the pancreas: a new entity? Am J Surg Pathol. 2002;26(6):698–704.

    Article  PubMed  Google Scholar 

  21. McEvoy MP, Rich B, Klimstra D, et al. Acinar cell cystadenoma of the pancreas in a 9-year-old boy. J Pediatr Surg. 2010;45(5):e7–9.

    Article  PubMed  Google Scholar 

  22. Cantrell BB, Cubilla AL, Erlandson RA, et al. Acinar cell cystadenocarcinoma of human pancreas. Cancer. 1981;47(2):410–6.

    Article  CAS  PubMed  Google Scholar 

  23. Huang Y, Cao YF, Lin JL, et al. Acinar cell cystadenocarcinoma of the pancreas in a 4-year-old child. Pancreas. 2006;33(3):311–2.

    Article  PubMed  Google Scholar 

  24. Perez EA, Gutierrez JC, Koniaris LG, et al. Malignant pancreatic tumors: incidence and outcome in 58 pediatric patients. J Pediatr Surg. 2009;44(1):197–203.

    Article  PubMed  Google Scholar 

  25. Ellerkamp V, Warmann SW, Vorwerk P, et al. Exocrine pancreatic tumors in childhood in Germany. Pediatr Blood Cancer. 2012;58(3):366–71.

    Article  PubMed  Google Scholar 

  26. Illyés G, Luczay A, Benyó G, et al. Cushing’s syndrome in a child with pancreatic acinar cell carcinoma. Endocr Pathol. 2007;18(2):95–102.

    Article  PubMed  Google Scholar 

  27. Matarazzo P, Tuli G, Tessaris D, et al. Cushing syndrome due to ectopic adrenocorticotropic hormone secretion in a 3-year-old child. J Pediatr Endocrinol Metab. 2011;24(3–4):219–22.

    PubMed  Google Scholar 

  28. Seth AK, Argani P, Campbell KA, et al. Acinar cell carcinoma of the pancreas: an institutional series of resected patients and review of the current literature. J Gastrointest Surg. 2008;12(6):1061–7.

    Article  PubMed  Google Scholar 

  29. Abraham SC, Wu TT, Hruban RH, et al. Genetic and immunohistochemical analysis of pancreatic acinar cell carcinoma: frequent allelic loss on chromosome 11p and alterations in the APC/beta-catenin pathway. Am J Pathol. 2002;160(3):953–62.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Jaffe R, Newman B. Pancreatic masses. In: Stocker JT, Askin FB, editors. Pathology of solid tumors in children. London/New York: Chapman & Hall Medical; 1998. p. 67–80.

    Google Scholar 

  31. Lüttges J, Stigge C, Pacena M, et al. Rare ductal adenocarcinoma of the pancreas in patients younger than age 40 years. Cancer. 2004;100(1):173–82.

    Article  PubMed  Google Scholar 

  32. Rebours V, Lévy P, Mosnier JF, et al. Pathology analysis reveals that dysplastic pancreatic ductal lesions are frequent in patients with hereditary pancreatitis. Clin Gastroenterol Hepatol. 2010;8(2):206–12.

    Article  PubMed  Google Scholar 

  33. Ito R, Kondo F, Yamaguchi T, et al. Pancreatic intraepithelial neoplasms in the normal appearing pancreas: on their precise relationship with age. Hepatogastroenterology. 2008;55(84):1103–6.

    PubMed  Google Scholar 

  34. Fanjiang G, Guelrud M, Gupta M, et al. Intraductal papillary-mucinous neoplasm of the pancreas in a 14-year-old. J Pediatr Gastroenterol Nutr. 2007;44(2):287–90.

    Article  PubMed  Google Scholar 

  35. Lau ST, Kim SS, Lee SL, et al. Mucinous cystadenoma of the pancreas in a one-year-old child. J Pediatr Surg. 2004;39(10):1574–5.

    Article  PubMed  Google Scholar 

  36. Mukai M, Takamatsu H, Noguchi H, et al. Post-pubertal mucinous cystadenoma of the pancreas. Pediatr Surg Int. 2001;17(7):545–8.

    Article  CAS  PubMed  Google Scholar 

  37. Oermann CM, Al-Salmi Q, Seilheimer DK, et al. Mucinous cystadenocarcinoma of the pancreas in an adolescent with cystic fibrosis. Pediatr Dev Pathol. 2005;8(3):391–6.

    Article  PubMed  Google Scholar 

  38. Montero M, Vázquez JL, Rihuete MA, et al. Serous cystadenoma of the pancreas in a child. J Pediatr Surg. 2003;38(9):E6–7.

    Article  PubMed  Google Scholar 

  39. Aggarwal G, Satsangi B, Shukla S, et al. Rare asymptomatic presentations of schwannomas in early adolescence: three cases with review of literature. Int J Surg. 2010;8(3):203–6.

    Article  PubMed  Google Scholar 

  40. Barresi L, Tarantino I, Curcio G, et al. Pancreatic cystic lymphangioma in a 6-year-old girl, diagnosed by endoscopic ultrasound (EUS) fine needle aspiration. Endoscopy. 2011;43(Suppl 2 UCTN):E61–2.

    Article  PubMed  Google Scholar 

  41. Menon BS, Juraida E, Mohamed M, et al. Pancreatic primitive neuroectodermal tumour associated with precocious puberty. Pediatr Blood Cancer. 2009;53(3):518–9.

    Article  PubMed  Google Scholar 

  42. Aftandilian CC, Friedmann AM. Burkitt lymphoma with pancreatic involvement. J Pediatr Hematol Oncol. 2010;32(8):e338–40.

    Article  PubMed  Google Scholar 

  43. Doi H, Ichikawa S, Hiraoka A, et al. Primitive neuroectodermal tumor of the pancreas. Intern Med. 2009;48(5):329–33.

    Article  PubMed  Google Scholar 

  44. Fraser CJ, Chan YF, Heath JA. Anaplastic large cell lymphoma of the pancreas: a pediatric case and literature review. J Pediatr Hematol Oncol. 2004;26(12):840–2.

    PubMed  Google Scholar 

  45. Eisenhuber E, Schoefl R, Wiesbauer P, et al. Primary pancreatic lymphoma presenting as acute pancreatitis in a child. Med Pediatr Oncol. 2001;37:53–4.

    Article  CAS  PubMed  Google Scholar 

  46. Langrehr JM, Bahra M, Kristiansen G, et al. Neuroendocrine tumor of the pancreas and bilateral adrenal pheochromocytomas. A rare manifestation of von Hippel-Lindau disease in childhood. J Pediatr Surg. 2007;42(7):1291–4.

    Article  PubMed  Google Scholar 

  47. Arva NC, Pappas JG, Bhatla T, et al. Well-differentiated pancreatic neuroendocrine carcinoma in tuberous sclerosis – case report and review of the literature. Am J Surg Pathol. 2012;36(1):149–53.

    Article  PubMed  Google Scholar 

  48. Kouvaraki MA, Shapiro SE, Cote GJ, et al. Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. World J Surg. 2006;30(5):643–53.

    Article  PubMed  Google Scholar 

  49. Ishikawa T, Itoh A, Kawashima H, et al. Usefulness of EUS combined with contrast-enhancement in the differential diagnosis of malignant versus benign and preoperative localization of pancreatic endocrine tumors. Gastrointest Endosc. 2010;71(6):951–9.

    Article  PubMed  Google Scholar 

  50. Anaye A, Mathieu A, Closset J, et al. Successful preoperative localization of a small pancreatic insulinoma by diffusion-weighted MRI. JOP. 2009;10(5):528–31.

    PubMed  Google Scholar 

  51. Bushnell DL, Baum RP. Endocrinol Metab Clin North Am. 2011;40(1):153–62, ix.

    Article  CAS  PubMed  Google Scholar 

  52. Okauchi Y, Nammo T, Iwahashi H, et al. Glucagonoma diagnosed by arterial stimulation and venous sampling (ASVS). Intern Med. 2009;48(12):1025–30.

    Article  PubMed  Google Scholar 

  53. Janem W, Sultan I, Ajlouni F, et al. Malignant insulinoma in a child. Pediatr Blood Cancer. 2010;55(7):1423–6.

    Article  PubMed  Google Scholar 

  54. Kattepura S, Das K, Correa MM, et al. Giant gastrinoma in a child: case report and review. Pediatr Surg Int. 2008;24(9):1083–5.

    Article  PubMed  Google Scholar 

  55. Schettini ST, Ribeiro RC, Facchin CG, et al. Gastrinoma in childhood: case report and update on diagnosis and treatment. Eur J Pediatr Surg. 2009;19(1):38–40.

    Article  CAS  PubMed  Google Scholar 

  56. Brenner RW, Sank LI, Kerner MB, et al. Resection of a vipoma of the pancreas in a 15-year-old girl. J Pediatr Surg. 1986;21(11):983–5.

    Article  CAS  PubMed  Google Scholar 

  57. Samal SC, Paul AC, Venkateswari S, et al. VIPoma of pancreas in a child. Indian J Gastroenterol. 2000;19(4):194–5.

    CAS  PubMed  Google Scholar 

  58. Stanley CA. Hyperinsulinism in infants and children. Pediatr Clin North Am. 1997;44:363–74.

    Article  CAS  PubMed  Google Scholar 

  59. Arnoux JB, de Lonlay P, Ribeiro MJ, et al. Congenital hyperinsulinism. Early Hum Dev. 2010;86(5):287–94.

    Article  CAS  PubMed  Google Scholar 

  60. Peranteau WH, Bathaii SM, Pawel B, et al. Multiple ectopic lesions of focal islet adenomatosis identified by positron emission tomography scan in an infant with congenital hyperinsulinism. J Pediatr Surg. 2007;42(1):188–92.

    Article  PubMed  Google Scholar 

  61. Bellanné-Chantelot C, Saint-Martin C, Ribeiro MJ, et al. ABCC8 and KCNJ11 molecular spectrum of 109 patients with diazoxide-unresponsive congenital hyperinsulinism. J Med Genet. 2010;47(11):752–9.

    Article  PubMed  Google Scholar 

  62. Laje P, Halaby L, Adzick NS, et al. Necrotizing enterocolitis in neonates receiving octreotide for the management of congenital hyperinsulinism. Pediatr Diabetes. 2010;11:142–7.

    Article  PubMed  Google Scholar 

  63. Palladino AA, Stanley CA. A specialized team approach to diagnosis and medical versus surgical treatment of infants with congenital hyperinsulinism. Semin Pediatr Surg. 2011;20(1):32–7.

    Article  PubMed  Google Scholar 

  64. Doppman JL, Miller DL, Chang R, et al. Insulinomas: localization with selective intraarterial injection of calcium. Radiology. 1991;178(1):237–41.

    Article  CAS  PubMed  Google Scholar 

  65. Brunelle F, Negre V, Barth MO, et al. Pancreatic venous samplings in infants and children with primary hyperinsulinism. Pediatr Radiol. 1989;19(2):100–3.

    Article  CAS  PubMed  Google Scholar 

  66. Adzick NS, Thornton PS, Stanley CA, et al. A multidisciplinary approach to the focal form of congenital hyperinsulinism leads to successful treatment by partial pancreatectomy. J Pediatr Surg. 2004;39(3):270–5.

    Article  PubMed  Google Scholar 

  67. Hoegerle S, Schneider B, Kraft A, et al. Imaging of a metastatic gastrointestinal carcinoid by F-18-DOPA positron emission tomography. Nuklearmedizin. 1999;38(4):127–30.

    CAS  PubMed  Google Scholar 

  68. Ribeiro MJ, De Lonlay P, Delzescaux T, et al. Characterization of hyperinsulinism in infancy assessed with PET and 18F-fluoro-L-DOPA. J Nucl Med. 2005;46(4):560–6.

    PubMed  Google Scholar 

  69. Hardy OT, Hernandez-Pampaloni M, Saffer JR, et al. Diagnosis and localization of focal congenital hyperinsulinism by 18F-fluorodopa PET scan. J Pediatr. 2007;150(2):140–5.

    Article  CAS  PubMed  Google Scholar 

  70. Laje P, Stanley CA, Palladino AA, et al. Pancreatic head resection and Roux-en-Y pancreaticojejunostomy for the treatment of the focal form of congenital hyperinsulinism. J Pediatr Surg. 2012;47(1):130–5.

    Article  PubMed  PubMed Central  Google Scholar 

  71. Bax NM, van der Zee DC, de Vroede M, et al. Laparoscopic identification and removal of focal lesions in persistent hyperinsulinemic hypoglycemia of infancy. Surg Endosc. 2003;17(5):833.

    Article  CAS  PubMed  Google Scholar 

  72. Al-Shanafey S. Laparoscopic vs open pancreatectomy for persistent hyperinsulinemic hypoglycemia of infancy. J Pediatr Surg. 2009;44(5):957–61.

    Article  PubMed  Google Scholar 

  73. Pierro A, Nah SA, et al. Surgical management of congenital hyperinsulinism of infancy. Semin Pediatr Surg. 2011;20(1):50–3.

    Article  PubMed  Google Scholar 

  74. Laje P, Stanley CA, Adzick NS. Intussusception after pancreatic surgery in children: a case series. J Pediatr Surg. 2010;45(7):1496–9.

    Article  PubMed  Google Scholar 

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Adzick, N.S., Laje, P. (2016). Pancreatic Neoplasms in Children. In: Carachi, R., Grosfeld, J. (eds) The Surgery of Childhood Tumors. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-48590-3_17

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