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Nonfunctioning endocrine tumor arising from intracranial ectopic pancreas associated with congenital brain malformation

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Case report

We report a case of a nonfunctioning pancreatic endocrine tumor arising from intracranial ectopic pancreatic tissue.


An 11-year-old girl was admitted to our hospital with a brain tumor. Hydrocephalus and brain malformation were apparent at birth. We first identified a mass-like lesion in the child’s brain at age 4 months. We monitored the lesion yearly by magnetic resonance imaging (MRI) until she reached age 11 years. Gadolinium-enhanced MRI showed the lesion to be a tumor, which was resected.


Examination of surgical specimens revealed a mature pancreatic tissue. We also identified monotonous neoplastic cells with round nuclei and positive immunoreactivity for synaptophysin, chromogranin A, and neurospecific enolase. However, these cells were negative for pancreatic endocrine markers. We diagnosed nonfunctioning pancreatic endocrine tumor arising from intracranial ectopic pancreatic tissue.


Migrating pancreatic elements may have induced brain malformation during embryonic development and subsequently become malignant.

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  1. Allison JW, Johnson JF 3rd, Barr LL, Warner BW, Stevenson RJ (1995) Induction of gastroduodenal prolapse by antral heterotopic pancreas. Pediatr Radiol 25:50–51

    Article  PubMed  CAS  Google Scholar 

  2. Cagirici U, Ozbaran M, Veral A, Posacioglu H (2001) Ectopic mediastinal pancreas. Eur J Cardiothorac Surg 19:514–515

    Article  PubMed  CAS  Google Scholar 

  3. Chen CH, Yang CC, Yeh YH, Chou DA, Kuo CL (2001) Ectopic pancreas located in the major duodenal papilla: case report and review. Gastrointest Endosc 53:121–123

    Article  PubMed  CAS  Google Scholar 

  4. Emerson L, Layfield LJ, Rohr LR, Dayton MT (2004) Adenocarcinoma arising in association with gastric heterotopic pancreas: case report and review of the literature. J Surg Oncol 87:53–57

    Article  PubMed  Google Scholar 

  5. Hayes-Jordan A, Idowu O, Cohen R (1998) Ectopic pancreas as the cause of gastric outlet obstruction in a newborn. Pediatr Radiol 28:868–870

    Article  PubMed  CAS  Google Scholar 

  6. Heitz PU, Bordi C, Komminoth P, Lechago J, Perren A, Centeno BA, Klimstra DS, Klöppel G, Dayal Y (2004) Pancreatic endocrine tumours. In: DeLellis RA, Lloyd RV, Heitz PU, Eng C (eds) Pathology and genetics of tumors of endocrine organs. World Health Organization classification of tumors. IARC, Lyon, pp 177–182

    Google Scholar 

  7. Ishikawa O, Ishiguro S, Ohhigashi H, Sasaki Y, Yasuda T, Imaoka S, Iwanaga T, Nakaizumi A, Fujita M, Wada A (1990) Solid and papillary neoplasm arising from an ectopic pancreas in the mesocolon. Am J Gastroenterol 85:597–601

    PubMed  CAS  Google Scholar 

  8. Jeong HY, Yang HW, Seo SW, Seong JK, Na BK, Lee BS, Song GS, Park HS, Lee HY (2002) Adenocarcinoma arising from an ectopic pancreas in the stomach. Endoscopy 34:1014–1017

    Article  PubMed  CAS  Google Scholar 

  9. Larsen WJ (1993) Human embryology. Churchill Livingstone, New York, pp 210–215, 397–400

    Google Scholar 

  10. Makhlouf HR, Almeida JL, Sobin LH (1999) Carcinoma in jejunal pancreatic heterotopia. Arch Pathol Lab Med 123:707–711

    PubMed  CAS  Google Scholar 

  11. Moore KL (1988) The developing human: clinically oriented embryology, 4th edn. Saunders, Philadelphia, pp 224–227

    Google Scholar 

  12. Nijs E, Callahan MJ, Taylor GA (2005) Disorders of the pediatric pancreas: imaging features. Pediatr Radiol 35:358–373

    Article  PubMed  Google Scholar 

  13. Özcan C, Çelik A, Güçlü C, Balık E (2002) A rare cause of gastric outlet obstruction in the newborn: pyloric ectopic pancreas. J Pediatr Surg 37:119–120

    Article  PubMed  Google Scholar 

  14. Perez-Ordonez B, Wesson DE, Smith CR, Asa SL (1996) A pancreatic cyst of the anterior mediastinum. Mod Pathol 9:210–214

    PubMed  CAS  Google Scholar 

  15. Rosai J (2004) Rosai and Ackerman’s surgical pathology, vol. 1. 9th edn. Mosby, Edinburgh, pp 649–650, 713

    Google Scholar 

  16. Roshe J, Del Buono E, Domenico D, Colturi TJ (1996) Anaplastic carcinoma arising in ectopic pancreas located in the distal esophagus. J Clin Gastroenterol 22:242–244

    Article  PubMed  CAS  Google Scholar 

  17. Tamura Y, Takahama M, Kushibe K, Taniguchi S (2005) Ectopic pancreas in the anterior mediastinum. Jpn J Thorac Cardiovasc Surg 53:498–501

    Article  PubMed  Google Scholar 

  18. Tornóczky T, Kálmán E, Jáksó P, Méhes G, Pajor L, Kajtár GG, Battyány I, Davidovics S, Sohail M, Krausz T (2001) Solid and papillary epithelial neoplasm arising in heterotopic pancreatic tissue of the mesocolon. J Clin Pathol 54:241–245

    Article  PubMed  Google Scholar 

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Correspondence to Hitoshi Tsugu.

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Tsugu, H., Oshiro, S., Kawaguchi, H. et al. Nonfunctioning endocrine tumor arising from intracranial ectopic pancreas associated with congenital brain malformation. Childs Nerv Syst 23, 1337–1340 (2007).

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