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.



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Narrow down the case reports



Total: 108 (papers)

   


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)		 PMID
(PMCID)
1
(51.4%)		 20497932
 Malignant somatostatinoma presenting with diabetic ketoacidosis and inhibitory syndrome: pathophysiologic considerations.
Theodoraki A, Khoo B, Hamda A, Grillo F, Meyer T, Bouloux PM.
Endocr Pract. 2010;16(5):835-7.
Cholelithiasis Anemia Diabetic ketoacidosis
SST
Adult Blood Glucose Complications of Diabetes Mellitus Diabetic Ketoacidosis Differential Diagnosis Females Homo sapiens Lymphatic Metastasis Pancreatic Neoplasm Somatostatinoma Syndrome
2
(48.0%)		 20300877
 Periampullary and duodenal neoplasms in neurofibromatosis type 1: two cases and an updated 20-year review of the literature yielding 76 cases.
Relles D, Baek J, Witkiewicz A, Yeo CJ.
J Gastrointest Surg. 2010;14(6):1052-61.
Jaundice Anemia
NF1
Adult Ampulla of Vater Common Bile Duct Neoplasms Duodenal Neoplasms Females Homo sapiens Male Middle Aged Neurofibromatosis 1
3
(37.5%)		 18303777
 [Malignant somatostatinoma (brief overview and a case review)].
Peskova M, Hvizd' R, Duskova J.
Rozhl Chir. 2007;86(12):643-7.
Anemia Abdominal pain
Homo sapiens Male Pancreatic Neoplasm Somatostatinoma
3
(37.5%)		 2897973
 Somatostatin-secreting islet cell tumor (somatostatinoma): suppression of growth hormone (GH) release induced by GH-releasing hormone.
Iguchi H, Kumagai S, Seo IH, Wakasugi H, Hara Y, Abe M.
J Clin Endocrinol Metab. 1988;67(1):206-10.
Anemia Dyspepsia
GH1 GHRH
Culture Techniques Females Gel Chromatography Growth Hormone Homo sapiens Immunohistochemistry Islet Cell Adenoma Pancreatic Neoplasm Somatostatinoma Somatotropin-Releasing Hormone
5
(32.9%)		 2855274
 Pancreatic somatostatinoma: presentation with recurrent episodes of severe hyperglycaemia and ketoacidosis.
Willcox PA, Immelman EJ, Barron JL, Klaff LJ, Harries-Jones EP, Hainsworth M, Millar RP.
Q J Med. 1988;68(255):559-71.
Weight loss Ketoacidosis Hepatomegaly
Diabetic Ketoacidosis Females Homo sapiens Hormones Hyperglycemia Islet Cell Adenoma Middle Aged Pancreatic Neoplasm Somatostatinoma
6
(32.0%)		 17438969
 [Somatostatinoma of the Vater's papilla in a patient with von Recklinghausen's disease].
Colovic R, Micev M, Grubor N, Radak V.
Vojnosanit Pregl. 2007;64(3):219-22.
Diabetes mellitus Jaundice
Ampulla of Vater Carcinoid Tumor Common Bile Duct Neoplasms Females Homo sapiens Middle Aged Neurofibromatosis 1 Somatostatinoma
7
(29.2%)		 21938182
(3168003)
 FNAC diagnosis of pancreatic somatostatinoma.
Dinesh U, Pervatikar SK, Rao R.
J Cytol. 2009;26(4):153-5.
Diabetes mellitus Cholelithiasis
7
(29.2%)		 17645474
 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.
Diabetes mellitus Cholelithiasis
NF1
Adult Duodenal Neoplasms Females Homo sapiens Jejunal Neoplasms Middle Aged Pancreatic Neoplasm Somatostatinoma
7
(29.2%)		 15849491
 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.
Diabetes mellitus Cholelithiasis
Cholangitis Differential Diagnosis Homo sapiens Islets of Langerhans Male Middle Aged Pancreatic Neoplasm Somatostatinoma
7
(29.2%)		 11606861
 Duodenal somatostatinoma presenting with complete somatostatinoma syndrome.
Green BT, Rockey DC.
J Clin Gastroenterol. 2001;33(5):415-7.
Diabetes mellitus Cholelithiasis
Ampulla of Vater Celiac Disease Cholelithiasis Diabetes Mellitus Duodenal Neoplasms Females Homo sapiens Middle Aged Neurofibromatosis 1 Somatostatinoma Syndrome
        

Phenotype(s) retrieved from Orphanet

    Total: 35

HPO ID Term Frequency
HP:0000819 Diabetes mellitus Frequent (79-30%)
HP:0001824 Weight loss Frequent (79-30%)
HP:0002014 Diarrhea Frequent (79-30%)
HP:0002017 Nausea and vomiting Frequent (79-30%)
HP:0002019 Constipation Frequent (79-30%)
HP:0002039 Anorexia Frequent (79-30%)
HP:0002240 Hepatomegaly Frequent (79-30%)
HP:0002570 Steatorrhea Frequent (79-30%)
HP:0002574 Episodic abdominal pain Frequent (79-30%)
HP:0002894 Neoplasm of the pancreas Frequent (79-30%)
HP:0004396 Poor appetite Frequent (79-30%)
HP:0004840 Hypochromic microcytic anemia Frequent (79-30%)
HP:0005609 Gallbladder dysfunction Frequent (79-30%)
HP:0012432 Chronic fatigue Frequent (79-30%)
HP:0100833 Neoplasm of the small intestine Frequent (79-30%)
HP:0001046 Intermittent jaundice Occasional (29-5%)
HP:0001406 Intrahepatic cholestasis Occasional (29-5%)
HP:0001438 Abnormality of abdomen morphology Occasional (29-5%)
HP:0001541 Ascites Occasional (29-5%)
HP:0002239 Gastrointestinal hemorrhage Occasional (29-5%)
HP:0005214 Intestinal obstruction Occasional (29-5%)
HP:0012334 Extrahepatic cholestasis Occasional (29-5%)
HP:0030145 Lack of bowel sounds Occasional (29-5%)
HP:0000820 Abnormality of the thyroid gland Very rare (4-1%)
HP:0000837 Increased circulating gonadotropin level Very rare (4-1%)
HP:0000845 Growth hormone excess Very rare (4-1%)
HP:0000870 Prolactin excess Very rare (4-1%)
HP:0001031 Subcutaneous lipoma Very rare (4-1%)
HP:0001578 None Very rare (4-1%)
HP:0002865 Medullary thyroid carcinoma Very rare (4-1%)
HP:0002893 Pituitary adenoma Very rare (4-1%)
HP:0002897 Parathyroid adenoma Very rare (4-1%)
HP:0003072 Hypercalcemia Very rare (4-1%)
HP:0008200 Primary hyperparathyroidism Very rare (4-1%)
HP:0008256 Adrenocortical adenoma Very rare (4-1%)


Phenotype(s) retrieved from case reports

    Total: 37

HPO ID Term # of case reports
HP:0001081 Cholelithiasis 9
HP:0002570 Steatorrhea 8
HP:0000819 Diabetes mellitus 7
HP:0002668 Paraganglioma 7
HP:0000952 Jaundice 4
HP:0001824 Weight loss 4
HP:0002014 Diarrhea 4
HP:0001943 Hypoglycemia 3
HP:0001953 Diabetic ketoacidosis 3
HP:0002666 Pheochromocytoma 3
HP:0003074 Hyperglycemia 3
HP:0030405 Pancreatic endocrine tumor 3
HP:0030731 Carcinoma 3
HP:0100570 Carcinoid tumor 3
HP:0002027 Abdominal pain 2
HP:0000843 Hyperparathyroidism 1
HP:0000957 Cafe-au-lait spot 1
HP:0001067 Neurofibromas 1
HP:0001903 Anemia 1
HP:0001952 Glucose intolerance 1
HP:0001993 Ketoacidosis 1
HP:0002018 Nausea 1
HP:0002028 Chronic diarrhea 1
HP:0002239 Gastrointestinal hemorrhage 1
HP:0002240 Hepatomegaly 1
HP:0002835 Aspiration 1
HP:0002897 Parathyroid adenoma 1
HP:0005864 Pseudoarthrosis 1
HP:0009792 Teratoma 1
HP:0012226 Ovarian teratoma 1
HP:0012334 Extrahepatic cholestasis 1
HP:0030358 Non-small cell lung carcinoma 1
HP:0032448 Achlorhydria 1
HP:0100008 Schwannoma 1
HP:0100732 Pancreatic fibrosis 1
HP:0410019 Epigastric pain 1
HP:0410281 Dyspepsia 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID