Somatostatinoma: clinico-pathological features of three cases and literature reviewed. 2008

Gabriella Nesi, and Tommaso Marcucci, and Carlos A Rubio, and Maria Luisa Brandi, and Francesco Tonelli
Department of Human Pathology and Oncology, University of Florence, Florence, Italy. gabriella.nesi@unifi.it

Somatostatinoma is a rare endocrine tumor that comprises around 1% of all gastroenteropancreatic endocrine neoplasms. This paper gives an updated review on somatostatinoma and describes three sporadic cases of somatostatinoma located in the pancreas, duodenum, and jejunum. Approximately 200 case histories of somatostatinoma have been published, with the duodenum being the most frequent site, followed by the pancreas. Somatostatinomas may be sporadic or associated with neurofibromatosis type 1, Multiple Endocrine Neoplasia type 1, and Von Hippel-Lindau syndromes. Functional somatostatinomas release excessive amounts of somatostatin suppressing gallbladder motility and inhibiting the secretory activity of various endocrine and exocrine cell types. A triad of mild diabetes mellitus, cholelithiasis, and diarrhea/steatorrhoea characterizes the somatostatinoma or 'inhibitory' syndrome. Non-functional somatostatinomas tend either to be asymptomatic or to present with obstructive symptoms. These tumors are often malignant and by the time they are detected, nearly two-thirds have already metastasized to the regional lymph nodes or the liver. A comparison between our three cases and those in the literature provides useful insights into the clinical management of these patients. Interestingly, the jejunal somatostatinoma described here is the second case ever reported.

UI MeSH Term Description Entries
D007580 Jejunal Neoplasms Tumors or cancer in the JEJUNUM region of the small intestine (INTESTINE, SMALL). Cancer of Jejunum,Cancer of the Jejunum,Cancer, Jejunal,Jejunal Cancer,Cancers, Jejunal,Jejunal Cancers,Jejunal Neoplasm,Jejunum Cancer,Jejunum Cancers,Neoplasm, Jejunal,Neoplasms, Jejunal
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
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
D004379 Duodenal Neoplasms Tumors or cancer of the DUODENUM. Cancer of Duodenum,Cancer of the Duodenum,Duodenal Cancer,Cancer, Duodenal,Duodenal Neoplasm,Duodenum Cancer,Neoplasm, Duodenal,Neoplasms, Duodenal
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
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

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