The hereditary forms of pancreatic neuroendocrine tumors.
Pancreatic endocrine tumors occur sporadically or in the setting of multiple endocrine neoplasia type I (MEN-1) or von-Hippel-Lindau disease. In the latter circumstances, the tumors are often multiple. This commentary addresses the differences in clinical, pathologic, and molecular features of MEN-1 and von Hippel-Lindau disease associated pancreatic endocrine tumors.
DeLellis
R A
RA
Department of Pathology, New York Presbyterian Hospital, NY, USA.
D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002893:Chromosomes, Human, Pair 3 / Q000235:genetics; D016147:Genes, Tumor Suppressor; D006801:Humans; D007150:Immunohistochemistry; D008025:Ligases; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications* / Q000235:genetics; D009363:Neoplasm Proteins / Q000235:genetics; D018358:Neuroendocrine Tumors / Q000150:complications* / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D011506:Proteins / Q000235:genetics; D011518:Proto-Oncogene Proteins; D025521:Tumor Suppressor Proteins; D044767:Ubiquitin-Protein Ligases; D051794:Von Hippel-Lindau Tumor Suppressor Protein; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000235:genetics / Q000378:metabolism / Q000473:pathology
D014408:Biomarkers, Tumor; C105373:MEN1 protein, human; D009363:Neoplasm Proteins; D011506:Proteins; D011518:Proto-Oncogene Proteins; D025521:Tumor Suppressor Proteins; D044767:Ubiquitin-Protein Ligases; D051794:Von Hippel-Lindau Tumor Suppressor Protein; D008025:Ligases; C497446:VHL protein, human