Somatostatinoma

Somatostatinoma (SSoma) is an extremely rare pancreatic neuroendocrine tumor or duodenal endocrine tumor (see these terms) that originates either in the pancreas (50%) or the gastrointestinal tract (50%) and mainly presents with non-specific symptoms of abdominal pain, weight loss, jaundice and diarrhea but, in approximately 20% of pancreatic cases, leads to a somatostatin hypersecretion syndrome (somatostatinoma syndrome) characterized by diabetes mellitus, cholelithiasis, steatorrhea and hypochlorhydria.

Diabetes mellitus

A group of abnormalities characterized by hyperglycemia and glucose intolerance.


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PMID (PMCID)
17645474
 FEMALE Adult
Somatostatinoma: clinico-pathological features of three cases and literature reviewed.
Nesi G, Marcucci T, Rubio CA, Brandi ML, Tonelli F.
J Gastroenterol Hepatol. 2008;23(4):521-6.
A triad of mild diabetes mellitus, cholelithiasis, and diarrhea/steatorrhoea characterizes the somatostatinoma or 'inhibitory' syndrome.
15849491
 MALE Middle Aged
Somatostatin-producing pancreatic endocrine carcinoma presented as relapsing cholangitis -- a case report.
Marakis G, Ballas K, Rafailidis S, Alatsakis M, Patsiaoura K, Sakadamis A.
Pancreatology. 2005;5(2-3):295-9.
Besides somatostatinoma syndrome, which is characterized by diabetes mellitus, steatorrhea and cholelithiasis, patients with somatostatin-producing endocrine tumors commonly complain of nonspecific symptoms such as vague abdominal pain, weight loss or changes in bowel habits.
11606861
 FEMALE Middle Aged
Duodenal somatostatinoma presenting with complete somatostatinoma syndrome.
Green BT, Rockey DC.
J Clin Gastroenterol. 2001;33(5):415-7.
Duodenal somatostatinomas are less common than, and are distinguished from, their pancreatic counterparts by a frequent association with type I neurofibromatosis, the presence of psammoma bodies, the less frequent presence of metastatic disease, and the absence of somatostatinoma syndrome (diabetes mellitus, steatorrhea, and cholelithiasis).
7494779
 FEMALE Middle Aged
Carcinoid syndrome due to a malignant somatostatinoma.
Ozbakir O, Kelestimur F, Ozturk F, Sozuer E, Unal A, Patiroglu TE, Guven K.
Postgrad Med J. 1995;71(841):695-8.
Cardinal manifestations of a somatostatinoma include gallstones, mild diabetes mellitus, steatorrhoea, diarrhoea and dyspepsia.
1974023
 MALE Adult
[Malignant somatostatinoma--diagnosis after 6 years].
Schaller P, Schweiger M, Stolte M, Lauer E.
Leber Magen Darm. 1990;20(3):152-6.
Early symptoms of malignant somatostatinoma with dyspeptic complaints, moderate diabetes mellitus and cholecystolithiasis are the reason of primary treatment of these patients by surgeons.
6314830
 FEMALE Adult
Pancreatic somatostatinoma.
Kelly TR.
Am J Surg. 1983;146(5):671-3.
The presence of cholelithiasis, steatorrhea, diabetes mellitus, and a pancreatic tumor initiated a diagnostic workup for somatostatinoma in a 43 year old black woman.
6102950
 FEMALE
Calcitonin-producing pancreatic somatostatinoma.
Galmiche JP, Chayvialle JA, Dubois PM, David L, Descos F, Paulin C, Ducastelle T, Colin R, Geffroy Y.
Gastroenterology. 1980;78(6):1577-83.
A pancreatic somatostatinoma metastatized to the liver was detected in a 70-yr-old woman presenting with chronic diarrhea, steatorrhea, pancreatic insufficiency, diabetes mellitus, and achlorhydria.