Journal of Gastrointestinal Cancer

, Volume 43, Supplement 1, pp 171–174 | Cite as

Concomitant Nonfunctional Pancreatic Neuroendocrine Tumor and Gastric GIST in a Patient Without Neurofibromatosis Type 1

  • Sven-Petter HaugvikEmail author
  • Bård Ingvald Røsok
  • Bjørn Edwin
  • Ivar Prydz Gladhaug
  • Øystein Mathisen
Case Report


Neuroendocrine tumors are rare neoplasms, stemming from the diffuse endocrine system and with potential of development in almost every organ, including the pancreas [17]. Pancreatic neuroendocrine tumors (pNET) are associated with different genetic abnormalities in regulatory pathways of the cell cycle [5, 9, 10, 15, 19].

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract [20] and originate from the interstitial cell of Cajal [11]. They represent a heterogenous group of neoplasms, which is characterized by genetic alterations in the oncogenes KIT and PDGFRA [7, 8]. These oncogenes are used as molecular targets of selective tyrosine kinase inhibitors (e.g. imatinib) in the medical treatment of GIST [4]. GIST show a realtively high incidence in patients with neurofibromatosis type 1 (NF-1) [21], which is the most frequent inherited disease in the Western world [22].

There are, to the best of our knowledge, only three...


Imatinib Octreotide Pancreatic Tail Pancreatic Neuroendocrine Tumor Gastric Gist 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



Computed tomography


Gastrointestinal stromal tumor


Neurofibromatosis type 1


Pancreatic neuroendocrine tumor



We would like to thank Prof. Sverre Heim, M.D., Ph.D., for analysing the cytogenetic data, Prof. Tor Jacob Eide, M.D., Ph.D., for performing the histopathology of the pNET and Dr. Jan Gunnar Fjeld, M.D., for analysing the data from the indium-111-labelled octreotide scintigraphy.

Competing Interests

The authors declare no conflict of interest.


  1. 1.
    Alabraba E et al. Pancreatic insulinoma co-existing with gastric GIST in the absence of neurofibromatosis-1. WJSO. 2009;7:18.Google Scholar
  2. 2.
    Bosman FT, Carneiro F, Hruban RH. WHO classification of tumours of the digestive system. Lyon: IARC; 2010.Google Scholar
  3. 3.
    Breiner JA et al. Loss of 14q and 22q in gastrointestinal stromal tumors (pacemaker cell tumors). Cancer Genet Cytogenet. 2000;120(2):111.PubMedCrossRefGoogle Scholar
  4. 4.
    Demetri GD et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. NEJM. 2002;347(7):472.PubMedCrossRefGoogle Scholar
  5. 5.
    Furlan D et al. Different molecular profiles characterize well-differentiated endocrine tumors and poorly differentiated endocrine carcinomas of the gastroenteropancreatic tract. Clin Cancer Res. 2004;10(3):947.PubMedCrossRefGoogle Scholar
  6. 6.
    Gunawan B et al. Biological and clinical significance of cytogenetic abnormalities in low-risk and high-risk gastrointestinal stromal tumors. Hum Pathol. 2002;33(3):316.PubMedCrossRefGoogle Scholar
  7. 7.
    Heinrich MC et al. PDGFRA activating mutations in gastrointestinal stromal tumors. Science. 2003;299(5607):708.PubMedCrossRefGoogle Scholar
  8. 8.
    Hirota S et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science. 1998;279(5350):577.PubMedCrossRefGoogle Scholar
  9. 9.
    Jiao Y et al. DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. Science. 2011;331(6021):1199.PubMedCrossRefGoogle Scholar
  10. 10.
    Kim do H et al. Allelic alterations in well-differentiated neuroendocrine tumors (carcinoid tumors) identified by genome-wide single nucleotide polymorphism analysis and comparison with pancreatic endocrine tumors. Genes Chromosomes Cancer. 2008;47(1):84.PubMedCrossRefGoogle Scholar
  11. 11.
    Kindblom LG et al. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol. 1998;152(5):1259.PubMedGoogle Scholar
  12. 12.
    Kover E et al. Dual tumours in the GI tract: synchronous and metachronous stromal (GIST) and epithelial/neuroendocrine neoplasms. Magy Onkol. 2004;48(4):315.PubMedGoogle Scholar
  13. 13.
    Maertens O et al. Molecular pathogenesis of multiple gastrointestinal stromal tumors in NF1 patients. Hum Mol Genet. 2006;15(6):1015.PubMedCrossRefGoogle Scholar
  14. 14.
    Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med. 2006;130(10):1466.PubMedGoogle Scholar
  15. 15.
    Missiaglia E et al. Pancreatic endocrine tumors: expression profiling evidences a role for AKT-mTOR pathway. J Clin Oncol. 2010;28(2):245.PubMedCrossRefGoogle Scholar
  16. 16.
    Pagogna S, Zanini N, Tomassetti P. Neuroendocrine tumor of the pancreas and GIST: is there a correlation? J Pancreas. 2004;6:501.Google Scholar
  17. 17.
    Rindi G, Wiedenmann B. Neuroendocrine neoplasms of the gut and pancreas: new insights. Nat Rev Endocrinol 2011. doi: 10.1038/nrendo.2011.120.
  18. 18.
    Rosok BI et al. Single-centre experience of laparoscopic pancreatic surgery. BJS. 2010;97(6):902.CrossRefGoogle Scholar
  19. 19.
    Tonnies H et al. Analysis of sporadic neuroendocrine tumours of the enteropancreatic system by comparative genomic hybridisation. Gut. 2001;48(4):536.PubMedCrossRefGoogle Scholar
  20. 20.
    van der Zwan SM, DeMatteo RP. Gastrointestinal stromal tumor: 5 years later. Cancer. 2005;104(9):1781.PubMedCrossRefGoogle Scholar
  21. 21.
    Wallace MR et al. Type 1 neurofibromatosis gene: identification of a large transcript disrupted in three NF1 patients. Science. 1990;249(4965):181.PubMedCrossRefGoogle Scholar
  22. 22.
    Ward BA, Gutmann DH. Neurofibromatosis 1: from lab bench to clinic. Pediatr Neurol. 2005;32(4):221.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Sven-Petter Haugvik
    • 1
    Email author
  • Bård Ingvald Røsok
    • 1
  • Bjørn Edwin
    • 1
    • 2
  • Ivar Prydz Gladhaug
    • 1
    • 3
  • Øystein Mathisen
    • 1
  1. 1.Department of Gastrointestinal SurgeryOslo University Hospital, RikshospitaletOsloNorway
  2. 2.Interventional Centre, Oslo University Hospital, RikshospitaletOsloNorway
  3. 3.Institute of Clinical MedicineUniversity of OsloOsloNorway

Personalised recommendations