Biomaterial Database

[Rare pancreatic endocrine tumors]. 2008

Robert Krysiak, and Bogustław Okopień, and Zbigniew Stanisław Herman

Klinika Chorób Wewnetrznych i Farmakologii Klinicznej, Slaski Uniwersytet Medyczny, Katowice. r.krysiak@pharmanet.com.pl

Functional pancreatic endocrine tumors other than gastrinoma and insulinoma are quite rare. Some of these tumors may be part of multiple endocrine neoplasia type one (MEN-1) syndrome or phakomatoses. Depending on their cell type, functional pancreatic endocrine tumors may cause distinct clinical endocrine syndromes, such as the 'glucagonoma syndrome', Verner-Morrison syndrome and the 'somatostatinoma syndrome'. The significant progress made in recent years, especially in the field of imaging procedures, has brought about great improvement in the identification and differentiation of these neoplasms. Currently, the only curative treatment for islet cell tumors is complete surgical resection. The medical treatment of endocrine pancreatic tumours consists of somatostatin analogues, chemotherapy, and interferon-alpha. The purpose of this manuscript is to provide an overview of the contemporary etiopathogenesis, diagnosis and treatment of rare pancreatic endocrine tumors.

UI	MeSH Term	Description	Entries
D010190	Pancreatic Neoplasms	Tumors or cancer of the PANCREAS. Depending on the types of ISLET CELLS present in the tumors, various hormones can be secreted: GLUCAGON from PANCREATIC ALPHA CELLS; INSULIN from PANCREATIC BETA CELLS; and SOMATOSTATIN from the SOMATOSTATIN-SECRETING CELLS. Most are malignant except the insulin-producing tumors (INSULINOMA).	Cancer of Pancreas,Pancreatic Cancer,Cancer of the Pancreas,Neoplasms, Pancreatic,Pancreas Cancer,Pancreas Neoplasms,Pancreatic Acinar Carcinoma,Pancreatic Carcinoma,Acinar Carcinoma, Pancreatic,Acinar Carcinomas, Pancreatic,Cancer, Pancreas,Cancer, Pancreatic,Cancers, Pancreas,Cancers, Pancreatic,Carcinoma, Pancreatic,Carcinoma, Pancreatic Acinar,Carcinomas, Pancreatic,Carcinomas, Pancreatic Acinar,Neoplasm, Pancreas,Neoplasm, Pancreatic,Neoplasms, Pancreas,Pancreas Cancers,Pancreas Neoplasm,Pancreatic Acinar Carcinomas,Pancreatic Cancers,Pancreatic Carcinomas,Pancreatic Neoplasm
D003969	Vipoma	A tumor that secretes VASOACTIVE INTESTINAL PEPTIDE, a neuropeptide that causes VASODILATION; relaxation of smooth muscles; watery DIARRHEA; HYPOKALEMIA; and HYPOCHLORHYDRIA. Vipomas, derived from the pancreatic ISLET CELLS, generally are malignant and can secrete other hormones. In most cases, Vipomas are located in the PANCREAS but can be found in extrapancreatic sites.	Diarrheogenic Tumor,Pancreatic Cholera,Verner-Morrison Syndrome,WDHA,WDHH,Watery Diarrhea Syndrome,Watery Diarrhea with Hypokalemic Alkalosis,Diarrheogenic Islet Cell Tumor,Pancreatic VIPoma,VIP-Secreting Tumor (VIPoma),Vasoactive Intestinal Peptide (VIP) Tumor,Vasoactive Intestinal Peptide-Producing Tumor,Vipoma Syndrome,WDHA Syndrome,Watery Diarrhea, Hypokalemia, and Achlorhydria Syndrome,Cholera, Pancreatic,Diarrheogenic Tumors,Pancreatic VIPomas,Syndrome, Verner-Morrison,Syndrome, Vipoma,Tumor, Diarrheogenic,Tumor, VIP-Secreting (VIPoma),Tumors, Diarrheogenic,Tumors, VIP-Secreting (VIPoma),VIP Secreting Tumor (VIPoma),VIP-Secreting Tumors (VIPoma),VIPoma, Pancreatic,VIPomas, Pancreatic,Vasoactive Intestinal Peptide Producing Tumor,Verner Morrison Syndrome,Vipomas,WDHA Syndromes
D005935	Glucagonoma	An almost always malignant GLUCAGON-secreting tumor derived from the PANCREATIC ALPHA CELLS. It is characterized by a distinctive migratory ERYTHEMA; WEIGHT LOSS; STOMATITIS; GLOSSITIS; DIABETES MELLITUS; hypoaminoacidemia; and normochromic normocytic ANEMIA.	Adenoma, alpha-Cell,alpha-Cell Tumor,Glucagonoma Syndrome,Adenoma, alpha Cell,Adenomas, alpha-Cell,Glucagonoma Syndromes,Glucagonomas,Syndrome, Glucagonoma,Syndromes, Glucagonoma,Tumor, alpha-Cell,Tumors, alpha-Cell,alpha Cell Tumor,alpha-Cell Adenoma,alpha-Cell Adenomas,alpha-Cell Tumors
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013005	Somatostatinoma	A SOMATOSTATIN-secreting tumor derived from the pancreatic delta cells (SOMATOSTATIN-SECRETING CELLS). It is also found in the INTESTINE. Somatostatinomas are associated with DIABETES MELLITUS; CHOLELITHIASIS; STEATORRHEA; and HYPOCHLORHYDRIA. The majority of somatostatinomas have the potential for METASTASIS.	Somatostatinomas
D035583	Rare Diseases	A large group of diseases which are characterized by a low prevalence in the population. They frequently are associated with problems in diagnosis and treatment.	Orphan Diseases,Disease, Orphan,Disease, Rare,Orphan Disease,Rare Disease