Glucagonoma

Glucagonoma is a rare, functioning type of pancreatic neuroendocrine tumor (PNET; see this term) that hypersecretes glucagon, leading to a syndrome comprised of necrolytic migratory erythema, diabetes mellitus, anemia, weight loss, mucosal abnormalities, thromboembolism, gastrointestinal and neuropsychiatric symptoms.



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Narrow down the case reports



Total: 234 (papers)

   


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)		 PMID
(PMCID)
2
(49.1%)		 2548427
 Response of glucagonomas to surgical excision and chemotherapy. Report of two cases and review of the literature.
Reyes-Govea J, Holm A, Aldrete JS.
Am Surg. 1989;55(8):523-7.
Diabetes mellitus Anemia Necrolytic migratory erythema
Females Glucagonoma Homo sapiens Islet Cell Adenoma Male Middle Aged Pancreatic Neoplasm
2
(49.1%)		 218426
 Metabolic studies and glucagon gel filtration pattern before and after surgery in a case of glucagonoma syndrome.
von Schenck H, Thorell JI, Berg J, Bojs G, Dymling JF, Hallengren B, Ljungberg O, Tibblin S.
Acta Med Scand. 1979;205(3):155-62.
Anemia Hypoglycemia Necrolytic migratory erythema
SST
Anemia Erythema Gel Chromatography Glossitis Glycosuria, Renal Homo sapiens Hypoglycemia Insulin Islet Cell Adenoma Male Nicotinic Acids Pancreatic Neoplasm Syndrome
2
(49.1%)		 194537
 Necrolytic migratory erythema. Distinctive dermatosis of the glucagonoma syndrome.
Kahan RS, Perez-Figaredo RA, Neimanis A.
Arch Dermatol. 1977;113(6):792-7.
Diabetes mellitus Anemia Necrolytic migratory erythema
Adult Differential Diagnosis Erythema Females Homo sapiens Islet Cell Adenoma Islets of Langerhans Male Middle Aged Pancreatic Neoplasm Syndrome
24
(45.6%)		 8928539
 [Paraneoplastic spastic tetraparesis in glucagonoma syndrome. Successful therapy with octreotide, dacarbazine and interferon-alpha].
Strohm WD.
Z Gastroenterol. 1996;34(7):438-45.
Diabetes mellitus Eczema Anemia Diarrhea
Adult Chemotherapy, Adjuvant Combined Modality Therapy Diagnostic Imaging Glucagonoma Homo sapiens Interferon-alpha Male Pancreatectomy Pancreatic Neoplasm Paraneoplastic Syndromes Quadriplegia
24
(45.6%)		 201809
 The glucagonoma syndrome and its management.
Kessinger A, Lemon HM, Foley JF.
J Surg Oncol. 1977;9(5):419-24.
Diabetes mellitus Skin rash Anemia Diarrhea
Adult Dermatitis Diabetes Mellitus Females Homo sapiens Islet Cell Adenoma Male Middle Aged Neoplasm Metastasis Pancreatic Neoplasm Syndrome
26
(44.3%)		 8729827
 [Necrolytic migrating erythema without glucagonoma].
Maillard H, Celerier P, Maisonneuve C, Forest JL, Blanchi A, Pasquiou C.
Ann Dermatol Venereol. 1995;122(11-12):786-8.
Cirrhosis Anemia Erythema
Adult Differential Diagnosis Erythema Fatal Outcome Glucagonoma Homo sapiens Liver Cirrhosis Male Necrosis Pancreatic Neoplasm Skin
27
(43.7%)		 23890147
 Necrolytic migratory erythema and glucagonoma rising from pancreatic head.
Tseng HC, Liu CT, Ho JC, Lin SH.
Pancreatology. 2013;13(4):455-7.
Diabetes mellitus Anemia Diarrhea
Females Glucagonoma Homo sapiens Middle Aged Necrolytic Migratory Erythema Pancreatic Neoplasm
28
(42.9%)		 20465606
 Glucagonoma syndrome and necrolytic migratory erythema.
Lobo I, Carvalho A, Amaral C, Machado S, Carvalho R.
Int J Dermatol. 2010;49(1):24-9.
Weight loss Anemia Necrolytic migratory erythema
Biopsy Erythema Glucagonoma Homo sapiens Male Necrosis Pancreatectomy Pancreatic Neoplasm Skin X-Ray Computed Tomography
28
(42.9%)		 16476361
 [Necrolytic migratory erythema associated with glucagonoma].
Mendoza-Guil F, Hernandez-Jurado I, Burkhardt P, Linares J, Naranjo R.
Actas Dermosifiliogr. 2005;96(3):175-8.
Weight loss Anemia Erythema
Erythema Females Glucagonoma Homo sapiens Middle Aged Necrosis Pancreatic Neoplasm
28
(42.9%)		 16435046
 Necrolytic migratory erythema associated with hyperglucagonemia and neuroendocrine hepatic tumors.
Marko PB, Miljkovic J, Zemljic TG.
Acta Dermatovenerol Alp Pannonica Adriat. 2005;14(4):161-4, 166.
Congestive heart failure Anemia Necrolytic migratory erythema
Antineoplastic Agents, Hormonal Dermatitis Erythema Homo sapiens Male Middle Aged Neuroendocrine Tumors Paraneoplastic Syndromes
        

Phenotype(s) retrieved from Orphanet

    Total: 42

HPO ID Term Frequency
HP:0002894 Neoplasm of the pancreas Very frequent (99-80%)
HP:0000206 Glossitis Frequent (79-30%)
HP:0000819 Diabetes mellitus Frequent (79-30%)
HP:0000988 Skin rash Frequent (79-30%)
HP:0000989 Pruritus Frequent (79-30%)
HP:0001824 Weight loss Frequent (79-30%)
HP:0001895 Normochromic anemia Frequent (79-30%)
HP:0001927 Acanthocytosis 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:0002574 Episodic abdominal pain Frequent (79-30%)
HP:0004396 Poor appetite Frequent (79-30%)
HP:0008066 Abnormal blistering of the skin Frequent (79-30%)
HP:0010280 Stomatitis Frequent (79-30%)
HP:0012432 Chronic fatigue Frequent (79-30%)
HP:0031181 Necrolytic migratory erythema Frequent (79-30%)
HP:0000716 Depressivity Occasional (29-5%)
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:0001907 Thromboembolism Occasional (29-5%)
HP:0002239 Gastrointestinal hemorrhage Occasional (29-5%)
HP:0002570 Steatorrhea 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:0030895 Abnormal gastrointestinal motility 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: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: 61

HPO ID Term # of case reports
HP:0031181 Necrolytic migratory erythema 79
HP:0001824 Weight loss 30
HP:0001903 Anemia 23
HP:0000819 Diabetes mellitus 21
HP:0010783 Erythema 14
HP:0002664 Neoplasm 10
HP:0030731 Carcinoma 8
HP:0001952 Glucose intolerance 6
HP:0010280 Stomatitis 6
HP:0000206 Glossitis 5
HP:0000988 Skin rash 5
HP:0030405 Pancreatic endocrine tumor 5
HP:0001953 Diabetic ketoacidosis 4
HP:0002024 Malabsorption 4
HP:0003074 Hyperglycemia 4
HP:0002014 Diarrhea 3
HP:0100825 Cheilitis 3
HP:0000843 Hyperparathyroidism 2
HP:0001513 Obesity 2
HP:0001733 Pancreatitis 2
HP:0001943 Hypoglycemia 2
HP:0002900 Hypokalemia 2
HP:0004395 Malnutrition 2
HP:0004398 Peptic ulcer 2
HP:0004936 Venous thrombosis 2
HP:0031500 Abdominal mass 2
HP:0500167 Hypergastrinemia 2
HP:0000100 Nephrotic syndrome 1
HP:0000648 Optic atrophy 1
HP:0000726 Dementia 1
HP:0000842 Hyperinsulinemia 1
HP:0000964 Eczema 1
HP:0001251 Ataxia 1
HP:0001285 Spastic tetraparesis 1
HP:0001399 Hepatic failure 1
HP:0001433 Hepatosplenomegaly 1
HP:0001873 Thrombocytopenia 1
HP:0001993 Ketoacidosis 1
HP:0002028 Chronic diarrhea 1
HP:0002039 Anorexia 1
HP:0002204 Pulmonary embolism 1
HP:0002570 Steatorrhea 1
HP:0002625 Deep venous thrombosis 1
HP:0002666 Pheochromocytoma 1
HP:0002668 Paraganglioma 1
HP:0002894 Neoplasm of the pancreas 1
HP:0003072 Hypercalcemia 1
HP:0003073 Hypoalbuminemia 1
HP:0003075 Hypoproteinemia 1
HP:0005206 Pancreatic pseudocyst 1
HP:0005213 Pancreatic calcification 1
HP:0008200 Primary hyperparathyroidism 1
HP:0010566 Hamartoma 1
HP:0011736 Primary hyperaldosteronism 1
HP:0012115 Hepatitis 1
HP:0030016 Dyspareunia 1
HP:0030318 Angular cheilitis 1
HP:0031002 Neuritis 1
HP:0032368 Acidemia 1
HP:0040192 APUdoma 1
HP:0100792 Acantholysis 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID