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.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Surveillance, Epidemiology and End Results (SEER) Program, National Cancer Institute, National Institutes of Health, Bethesda. www.seer.cancer.gov .
Classification of tumors of the digestive system, 4th ed. Lyon: International Agency for Research on Cancer, World Health Organization: 2010.
Cavallini A, Falconi M, Bortesi L, et al. Pancreatoblastoma in adults: a review of the literature. Pancreatology. 2009;9(1–2):73–80.
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.
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.
Kletter GB, Sweetser DA, Wallace SF, et al. Adrenocorticotropin-secreting pancreatoblastoma. J Pediatr Endocrinol Metab. 2007;20(5):639–42.
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.
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.
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.
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.
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.
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.
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.
Tipton SG, Smyrk TC, Sarr MG, et al. Malignant potential of solid pseudopapillary neoplasm of the pancreas. Br J Surg. 2006;93:733e7.
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.
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.
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.
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.
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.
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.
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.
Cantrell BB, Cubilla AL, Erlandson RA, et al. Acinar cell cystadenocarcinoma of human pancreas. Cancer. 1981;47(2):410–6.
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.
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.
Ellerkamp V, Warmann SW, Vorwerk P, et al. Exocrine pancreatic tumors in childhood in Germany. Pediatr Blood Cancer. 2012;58(3):366–71.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Mukai M, Takamatsu H, Noguchi H, et al. Post-pubertal mucinous cystadenoma of the pancreas. Pediatr Surg Int. 2001;17(7):545–8.
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.
Montero M, Vázquez JL, Rihuete MA, et al. Serous cystadenoma of the pancreas in a child. J Pediatr Surg. 2003;38(9):E6–7.
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.
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.
Menon BS, Juraida E, Mohamed M, et al. Pancreatic primitive neuroectodermal tumour associated with precocious puberty. Pediatr Blood Cancer. 2009;53(3):518–9.
Aftandilian CC, Friedmann AM. Burkitt lymphoma with pancreatic involvement. J Pediatr Hematol Oncol. 2010;32(8):e338–40.
Doi H, Ichikawa S, Hiraoka A, et al. Primitive neuroectodermal tumor of the pancreas. Intern Med. 2009;48(5):329–33.
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.
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.
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.
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.
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.
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.
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.
Bushnell DL, Baum RP. Endocrinol Metab Clin North Am. 2011;40(1):153–62, ix.
Okauchi Y, Nammo T, Iwahashi H, et al. Glucagonoma diagnosed by arterial stimulation and venous sampling (ASVS). Intern Med. 2009;48(12):1025–30.
Janem W, Sultan I, Ajlouni F, et al. Malignant insulinoma in a child. Pediatr Blood Cancer. 2010;55(7):1423–6.
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.
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.
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.
Samal SC, Paul AC, Venkateswari S, et al. VIPoma of pancreas in a child. Indian J Gastroenterol. 2000;19(4):194–5.
Stanley CA. Hyperinsulinism in infants and children. Pediatr Clin North Am. 1997;44:363–74.
Arnoux JB, de Lonlay P, Ribeiro MJ, et al. Congenital hyperinsulinism. Early Hum Dev. 2010;86(5):287–94.
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.
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.
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.
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.
Doppman JL, Miller DL, Chang R, et al. Insulinomas: localization with selective intraarterial injection of calcium. Radiology. 1991;178(1):237–41.
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.
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.
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.
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.
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.
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.
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.
Al-Shanafey S. Laparoscopic vs open pancreatectomy for persistent hyperinsulinemic hypoglycemia of infancy. J Pediatr Surg. 2009;44(5):957–61.
Pierro A, Nah SA, et al. Surgical management of congenital hyperinsulinism of infancy. Semin Pediatr Surg. 2011;20(1):50–3.
Laje P, Stanley CA, Adzick NS. Intussusception after pancreatic surgery in children: a case series. J Pediatr Surg. 2010;45(7):1496–9.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
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
Download citation
DOI: https://doi.org/10.1007/978-3-662-48590-3_17
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-662-48588-0
Online ISBN: 978-3-662-48590-3
eBook Packages: MedicineMedicine (R0)