Molecular Pathology and Diagnostics of Pancreatic Endocrine Neoplasms

  • Aejaz NasirEmail author
  • Jonathan R. Strosberg
  • Larry K. Kvols
  • Jalil Muhammad
  • Nelly A. Nasir
  • Omie Mills
  • Domenico Coppola
Part of the Cancer Growth and Progression book series (CAGP, volume 16)


Pancreatic Endocrine Neoplasms (PENs) are a group of rare tumors thought to arise from the endocrine cells of the pancreas. These tumors may be functional (hormone producing), or non functional. They have a wide range of presenting symptoms. Recent research efforts have shown the complex biology of these tumors, and have started to uncover the molecular alterations responsible for the genesis of these neoplasms. In this chapter we give an overview of the molecular tests available to detect such alterations, and of their diagnostic and prognostic significance.


Pancreatic endocrine neoplasms Pancreatic endocrine tumors/carcinomas Classifications of PENS Molecular genetic Islets of Langerhans 



Activator protein-1


Basic fibroblast growth factor


Clinically localized pancreatic endocrine tumors


Candidate progression genes


Estrogen receptor gene


Fragile histidine triad


Human MutL homologue


Insulin-like growth factor


Insulin-like growth factor binding protein 3




Loss of heterozygosity


Metastasis-associated gene


Monohormonal endocrine cell clusters


Multiple endocrine neoplasia type 1


Metastatic primary


Mammalian target of rapamycin


Neuofibromatosis type 1


Nerve growth factor


Pancreatic endocrine carcinomas


Pancreatic endocrine neoplasm


Pancreatic endocrine tumors


Parathyroid adenoma-related protein


Succinate dehydrogenase subunit D




G-protein-coupled transmembrane receptors


Transforming growth factor


Tuberous sclerosis genes 1 & 2


von Hippel-Lindau genes



The authors would like to acknowledge the excellent support from Brenda Barrow and Rasa Hamilton of Moffitt Cancer Center, Tampa, FL, USA.


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Copyright information

© Springer Science+Business Media Dordrecht 2014

Authors and Affiliations

  • Aejaz Nasir
    • 1
    Email author
  • Jonathan R. Strosberg
    • 2
  • Larry K. Kvols
    • 2
  • Jalil Muhammad
    • 3
  • Nelly A. Nasir
    • 4
  • Omie Mills
    • 5
  • Domenico Coppola
    • 2
    • 5
    • 6
  1. 1.Diagnostic & Experimental Pathology & Tailored Therapeutics ProgramEli Lilly & CompanyIndianapolisUSA
  2. 2.GI Tumor Program, Neuroendocrine Research DivisionH. Lee Moffitt Cancer Center and Research InstituteTampaUSA
  3. 3.School of MedicineGeorge Mason UniversityFalls ChurchUSA
  4. 4.Department of Pathology & Laboratory Medicine, Sir Mortimer Jewish General HospitalMcGill UniversityMontrealCanada
  5. 5.Department of Pathology & Cell Biology, College of MedicineUniversity of South FloridaTampaUSA
  6. 6.Department of Anatomic PathologyH. Lee Moffitt Cancer Center and Research InstituteTampaUSA

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