Molecular Pathology of Pancreatic Endocrine Tumors
The molecular biology of Pancreatic Endocrine Tumors (PETs) carcinogenesis is poorly understood and is generally different from that of exocrine pancreatic neoplasms. PETs represent a rare group of neoplasms with heterogenous clinico-pathological features
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Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 172
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Inherited Pancreatic Endocrine Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 172 Multiple Endocrine Neoplasia Type I (MEN-I) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173 Von Hippel-Lindau Disease (VHL) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175 Von Recklinghausen’s Disease or Neurofibromatosis Type 1 (NF-1) . . . . . . . . . . . . . . . 176 Tuberous Sclerosis Complex (TSC) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176
3 3.1 3.2 3.3
Genetic Instability in Sporadic Pancreatic Endocrine Tumors . . . . . . . . . . . . . . . . . . . . 177 Genome Wide Studies in Sporadic PETs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 177 Prognostic Relevance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 178 Final Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185
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Genetic Alterations of Oncogenes and Tumor Suppressor Genes, and Expression of Growth Factors and Their Receptors . . . . . . . . . . . . . . . . . . . . . . . . . . 185 Oncogenes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185 Tumor Suppressor Genes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 186 Growth Factors and Their Receptors (Receptor Tyrosine Kinases) . . . . . . . . . . . . . . . . . 187 The (PI3K)/Protein Kinase B/AKT/mTOR Pathway . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 188
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Microarray Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 188
J. P. Neoptolemos, R. Urrutia, J. L. Abbruzzese, M. W. Bu¨chler (eds.), Pancreatic Cancer, DOI 10.1007/978-0-387-77498-5_7, # Springer Science+Business Media, LLC 2010
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Molecular Pathology of Pancreatic Endocrine Tumors
Abstract: The molecular biology of Pancreatic Endocrine Tumors (PETs) carcinogenesis is poorly understood and is generally different from that of exocrine pancreatic neoplasms. PETs represent a rare group of neoplasms with heterogenous clinico-pathological features. They are generally sporadic but can also arise within very rare hereditary syndromes, such as Multiple Endocrine Neoplasia type I (MEN-I), von Hippel-Lindau disease (VHL), Neurofibromatosis type 1 (NF1) and Tuberous Sclerosis Complex (TSC). In these syndromes although a specific genotype/phenotype association with PETs ha
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