KEGG   DISEASE: H00045Help
Entry
H00045                      Disease                                

Name
Malignant islet cell carcinoma
Description
Pancreatic endocrine neoplasms (PENs), commonly referenced as islet cell tumors, are relatively uncommon tumors. The most common type is of beta-cell origin and is known as insulinoma, which can be either benign or malignant. The majority of insulinomas arise sporadically, but a small proportion develop as part of the hereditary multiple endocrine neoplasia type 1 (MEN1) syndrome. MEN1 is an autosomal dominant inherited syndrome that occurs due to inactivating mutations of the MEN1 gene locus, coding for a tumor-suppressor protein, menin. About sporadic PENs, limited information regarding underlying genetic and molecular alterations is known. Alterations of the p16 gene, caused by deletion or methylation, have been identified in 42-58% of sporadic PENs, although these findings are controversial and may vary significantly based on PEN subtype.
Category
Cancer
BRITE hierarchy
Gene
MEN1 (mutation) [HSA:4221] [KO:K14970]
p16/INK4A (deletion, methylation) [HSA:1029] [KO:K06621]
Marker
CK19 (cytokeratin) [HSA:3880]
Ki-67 [HSA:4288]
Drug
Omeprazole [DR:D00455 D01207 D05259 D05261]
Lansoprazole [DR:D00355]
Diazoxide [DR:D00294]
Octreotide acetate (Sandostatin) [DR:D02250]
Streptozocin [DR:D05932]
Doxorubicin [DR:D03899 D01275]
Interferon alpha [DR:D00745 D02745 D03305 D04552 D04553]
Comment
ICD-O: 8150/3, Tumor type: Well-differentiated endocrine carcinoma
ICD-O: 8151/3, Tumor type: Insulin-producing (insulinoma)
ICD-O: 8153/3, Tumor type: Gastrin-producing (gastrinoma)
ICD-O: 8155/3, Tumor type: VIP-producing (VIPoma)
Other DBs
ICD-10: 
MeSH: 
Reference
PMID:11733227 (description, gene, tumor type)
  Authors
Pelengaris S, Khan M
  Title
Oncogenic co-operation in beta-cell tumorigenesis.
  Journal
Endocr Relat Cancer 8:307-14 (2001)
Reference
PMID:16357560 (gene, tumor type)
  Authors
House MG, Schulick RD.
  Title
Endocrine tumors of the pancreas.
  Journal
Curr Opin Oncol 18:23-9 (2006)
Reference
PMID:16253899 (gene)
  Authors
Oberg K, Eriksson B.
  Title
Endocrine tumours of the pancreas.
  Journal
Best Pract Res Clin Gastroenterol 19:753-81 (2005)
Reference
PMID:17394496 (marker)
  Authors
La Rosa S, Rigoli E, Uccella S, Novario R, Capella C.
  Title
Prognostic and biological significance of cytokeratin 19 in pancreatic endocrine tumours.
  Journal
Histopathology 50:597-606 (2007)
Reference
PMID:9495193 (marker)
  Authors
Lloyd RV.
  Title
Utility of Ki-67 as a prognostic marker in pancreatic endocrine neoplasms.
  Journal
Am J Clin Pathol 109:245-7 (1998)
Reference
(drug)
  Authors
Skeel RT (ed).
  Title
Handbook of Cancer Chemotherapy, Sixth Edition
  Journal
Lippincott Williams & Wilkins (2003)

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