| Entry |
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| Name |
Pancreatic neuroendocrine tumor |
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| Description |
Pancreatic neuroendocrine tumors (PNETs), also known as islet cell tumors, are rare neoplasms that arise in the endocrine tissues of the pancreas. They may occur sporadically or in association with a genetic syndrome, such as multiple endocrine neoplasia type 1 (MEN1), Von Hippel-Landau (VHL) syndrome, neurofibromatosis type 1, or tuberous sclerosis. The most frequent genetic alterations in PNET occur in MEN1 (Multiple Endocrine Neoplasia-1 Gene), DAXX/ATRX (Death-Domain Associated Protein/Mental Retardation Syndrome X-Linked Genes) and the mTOR pathway (Mammalian Target of Rapamycin). A germline mutation in the MEN1 tumor suppressor gene causes MEN1, the above-mentioned autosomal dominant hereditary syndrome. Mutations of DAXX and ATRX are common and related to altered telomeres but not to prognosis.
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| Category |
Cancer
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| Brite |
Human diseases [BR:br08402]
Cancers
Cancers of the digestive system
H00045 Pancreatic neuroendocrine tumor
Human diseases in ICD-11 classification [BR:br08403]
02 Neoplasms
Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
Malignant neoplasms of digestive organs
2C10 Malignant neoplasm of pancreas
H00045 Pancreatic neuroendocrine tumor
Tumor markers [br08442.html]
H00045
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| Gene |
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| Drug |
Lanreotide acetate [DR: D04666]
Streptozocin [DR: D05932]
Everolimus [DR: D02714]
Sunitinib malate [DR: D06402]
Lutetium (177Lu) oxodotreotide [DR: D11033] (somatostatin receptor-positive)
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| Comment |
ICD-O-3: 8151/3 Insulinoma, malignant (C25._)
ICD-O-3: 8153/3 Gastrinoma, malignant
ICD-O-3: 8155/3 Vipoma, malignant
ICD-O-3: 8152/3 Glucagonoma, malignant (C25._)
ICD-O-3: 8156/3 Somatostatinoma, malignant
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| Other DBs |
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| Reference |
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| Authors |
Viudez A, De Jesus-Acosta A, Carvalho FL, Vera R, Martin-Algarra S, Ramirez N |
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| Title |
Pancreatic neuroendocrine tumors: Challenges in an underestimated disease. |
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| Journal |
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| Reference |
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| Authors |
Capurso G, Archibugi L, Delle Fave G |
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| Title |
Molecular pathogenesis and targeted therapy of sporadic pancreatic neuroendocrine tumors. |
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| Journal |
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| Reference |
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| Authors |
Halperin DM, Kulke MH, Yao JC |
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| Title |
A tale of two tumors: treating pancreatic and extrapancreatic neuroendocrine tumors. |
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| Journal |
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| Reference |
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| Authors |
Rustagi T, Farrell JJ |
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| Title |
Endoscopic diagnosis and treatment of pancreatic neuroendocrine tumors. |
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| Journal |
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| Reference |
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| Authors |
Karakaxas D, Gazouli M, Liakakos T, Vaiopoulou A, Apessou D, Papaparaskeva K, Patapis P, Dervenis C |
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| Title |
Pancreatic neuroendocrine tumors: current opinions on a rare, but potentially curable neoplasm. |
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| Journal |
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| Reference |
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| Authors |
Ro C, Chai W, Yu VE, Yu R |
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| Title |
Pancreatic neuroendocrine tumors: biology, diagnosis,and treatment. |
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| Journal |
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| Reference |
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| Authors |
Capurso G, Festa S, Valente R, Piciucchi M, Panzuto F, Jensen RT, Delle Fave G |
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| Title |
Molecular pathology and genetics of pancreatic endocrine tumours. |
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| Journal |
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| Reference |
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| Authors |
Grozinsky-Glasberg S, Mazeh H, Gross DJ |
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| Title |
Clinical features of pancreatic neuroendocrine tumors. |
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| Journal |
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| LinkDB |
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