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.

Also Known As:

Networked: 317 relevant articles (11 outcomes, 14 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasm Metastasis (Metastasis)
2.	Neoplasms (Cancer)
3.	Gastrinoma
4.	Carcinoid Tumor (Carcinoid)
5.	Insulinoma

Experts

1.	Komoto, Izumi: 3 articles (10/2013 - 11/2002)
2.	Imamura, Masayuki: 3 articles (10/2013 - 11/2002)
3.	Fujikura, Junji: 3 articles (01/2013 - 11/2002)
4.	Hosoda, Kiminori: 3 articles (01/2013 - 11/2002)
5.	Doi, Ryuichiro: 3 articles (01/2013 - 11/2002)
6.	Nakao, Kazuwa: 3 articles (01/2013 - 11/2002)
7.	Tomita, Tsutomu: 3 articles (01/2013 - 11/2002)
8.	Tomita, T: 3 articles (05/2006 - 04/2000)
9.	Anlauf, M: 2 articles (06/2014 - 06/2011)
10.	Linder, Keith E: 2 articles (08/2013 - 10/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Glucagonoma:

1.	Octreotide (Sandostatin)FDA LinkGeneric 06/01/2005 - "She was operated on, and glucagonoma was detected and the long-acting, repeatable, octreotide treatment was started. " 04/01/2005 - "We present the case of a patient with malignant glucagonoma treated with surgery and octreotide, which manifested with skin lesions. " 02/01/2000 - "In this report, imaging of a glucagonoma with In-111 DTPA-D-PHE1 octreotide scintigraphy is described in a 51-year-old woman examined for a large palpable abdominal mass." 01/01/1989 - "Octreotide (SMS 201-995) in the treatment of metastatic glucagonoma: report of one case and review of the literature." 10/01/2010 - "In conclusion, subcutaneous octreotide injections were beneficial in this dog with glucagonoma-associated NME. "
2.	Dacarbazine (DIC)FDA LinkGeneric 10/01/1987 - "Although there is no wide experience in treatment, the literature seems to indicate that DTIC should be the drug of choice in treating metastatic glucagonoma. " 03/17/1984 - "Successful treatment of metastatic glucagonoma with dacarbazine." 12/01/1982 - "Glucagonoma and dacarbazine." 01/01/2000 - "[Chemoembolization followed by systemic chemotherapy with dacarbazine for a metastatic glucagonoma]." 09/01/1980 - "We conclude that dacarbazine is an effective mode of chemotherapy for malignant glucagonoma."
3.	Glucagon (Glukagon)FDA Link 06/01/2012 - "Serum glucagon levels were elevated to 54,405 pg/mL and a preoperative diagnosis of glucagonoma was made. " 01/01/2010 - "This case report demonstrates that the differential immunohistochemical reactivities of Siemens' Double Antibody Glucagon compared to DakoCytomation's Polyclonal Rabbit Anti-Human Glucagon allow for pathologic distinction of enteral versus pancreatic glucagonoma. " 01/01/2009 - "Glucagonoma is an uncommon disease, a neuroendocrine tumour that develops from glucagon-producing pancreatic cells. " 11/01/2007 - "In the early stage of a glucagonoma, however, the plasma glucagon level may be only modestly elevated and may still be susceptible to normal negative feedback inhibition. " 05/01/2007 - "In glucagonoma cells transfected with an Nkx6.1-encoding vector, the glucagon promoter activity was reduced by 65%. "
4.	Somatostatin (Somatotropin Release-Inhibiting Factor)IBA 07/01/1990 - "Somatostatin analogue and tissue cultures in the study of a human malignant glucagonoma." 10/01/2010 - "This somatostatin analogue could be a valuable option to treat canine patients with non-resectable or relapsing pancreatic glucagonoma-associated NME." 10/01/2002 - "The original presentation and benefit of a new, long-acting somatostatin analog for the treatment of inoperable glucagonoma are discussed." 10/01/2002 - "[Clinical response of an atypical glucagonoma treated with a long-acting somatostatin analog]." 02/01/1988 - "Furthermore, it has been shown previously that somatostatin rapidly improves skin lesions in glucagonoma patients. "
5.	Messenger RNA (mRNA)IBA 03/01/1994 - "In two cases of glucagonoma and its metastatic lymph nodes in one case, all the SSTR subtype mRNAs except SSTR5 mRNA were expressed. " 01/01/2013 - "Expression of GPR119 mRNA in fresh samples of normal human pancreas (n=19) and pancreatic islets (n=3) and in insulinomas (n=2) and glucagonomas (n=2), all collected at surgery, was compared with the mRNA expression of various receptors highly expressed and operative in human pancreatic islets. " 12/01/2003 - "Although the level of isl1-beta mRNA is much lower than that of isl1-alpha, isl1-beta is preferentially expressed in murine insulinoma cell lines but not in glucagonoma cell line. " 01/15/1998 - "Transplantable rat glucagonomas cause acute onset of severe anorexia and adipsia despite highly elevated NPY mRNA levels in the hypothalamic arcuate nucleus." 01/01/2013 - "GPR119 mRNA was also abundant in two cases of insulinoma and two cases of glucagonoma, but was undetectable in a pancreatic acinar cell tumor. "
6.	HormonesIBA 03/01/1992 - "The alpha-chain of the chorionic gonadotrophin hormone has limited value in the diagnosis of malignant glucagonoma. " 06/01/1988 - "[An autopsy case of glucagonoma associated with production of several hormones]." 08/01/1985 - "Multiple hormone secretion by a human pancreatic glucagonoma in culture." 09/01/2003 - "In particular, extrapancreatic gastrinoma, pancreatic glucagonoma, and mixed hormone-producing tumors in islets were observed. " 06/01/1999 - "Glucagonoma and somatostatinoma are tumors which produce the respective hormone. "
7.	GastrinsIBA 05/01/1987 - "In gastrinomas, SMS decreased plasma gastrin in all but one patient, inhibited the residual gastric acid secretion under H2-blockers and improved diarrhea; in the glucagonoma patient, glucagonemia decreased and skin lesions disappeared. " 05/01/2000 - "In the two patients with Zollinger-Ellison syndrome/multiple endocrine neoplasma type 1 and in the patient with glucagonoma, serum gastrin and plasma glucagon, respectively, decreased considerably. " 09/01/1998 - "A marked reduction in symptoms, and in plasma glucagon and serum gastrin levels, was also observed in patients with glucagonoma and gastrinoma. " 10/01/1985 - "With immunocytochemical staining, all six insulinomas, one mixed insulinoma-glucagonoma, and four gastrinomas were positive for insulin, insulin and glucagon, and gastrin, respectively. " 05/01/2010 - "Histological/immunohistochemical diagnosis was somatostatin-producing tumour in the first patient, oncocytic endocrine tumour positive for neurone-specific enolase and focally for chromogranin in the second patient, glucagonoma and pancreatic polypeptide-producing endocrine pancreatic tumour in the third patient, and gastrin, somatostatin, calcitonin, insulin and adrenocorticotropic hormone (ACTH)-producing tumour in the fourth. "
8.	Fluorouracil (Carac)FDA LinkGeneric 09/01/1992 - "Metastatic glucagonoma: clinical response to a combination of 5-fluorouracil and alpha-interferon." 01/01/2010 - "[Dramatic efficacy of chemotherapy with 5-fluorouracil and dacarbazine in a patient with metastatic glucagonoma and cardiac insufficiency]." 01/01/1983 - "We present a patient with a metastasizing pancreatic glucagonoma, in whom treatment with neither DTIC nor with the combination of streptozotocin and 5-fluorouracil resulted in any noticeable improvement." 01/01/2004 - "A diagnosis of metastatic glucagonoma was established and therapy with streptozocin, 5-FU, insulin and synthetic somatostatin analogs was initiated. "
9.	WDHA syndromeIBA 01/01/2000 - "Some of these substances cause a specific clinical syndrome: carcinoid, Zollinger-Ellison, hyperglycemic, glucagonoma and WDHA syndrome. " 10/01/1999 - "Each of these substances cause a specific clinical syndrome: carcinoid, Zollinger-Ellison, hyperglycaemic, glucagonoma and WDHA syndrome. " 06/01/1982 - "On the other hand, ultrasonography was very effective for evaluating the nonbeta cell tumors, detecting both primary pancreatic neoplasms and hepatic metastases in patients with glucagonomas, somatostatinoma, non-functioning tumors, and the WDHA syndrome. " 01/01/1989 - "Of the 84 patients, 23 had insulinomas, 25 gastrinomas, 20 nonfunctioning tumors, 14 the WDHA syndrome, 1 somatostatinoma and 1 glucagonoma. "
10.	thymidine 5'-triphosphate (TTP)IBA 10/01/2015 - "The hematological symptoms resolved completely after the procedure and 3 years later, the patient is well with no sign of recurrence of TTP or glucagonoma. " 10/01/2015 - "To our knowledge, this represents the first documented case of a non-secreting benign pancreatic neuroendocrine tumor (glucagonoma) associated with TTP that is refractory to standard treatment."

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 08/01/1989 - "Response of glucagonomas to surgical excision and chemotherapy. " 03/01/1987 - "Transcatheter arterial embolization was used to treat a patient with glucagonoma metastatic to the liver after chemotherapy failed. " 12/19/1987 - "We describe the 4-year follow-up of an endocrine tumour of the pancreas (vipoma-glucagonoma) treated with chemotherapy. " 01/01/2010 - "We report here an observation of a patient who was treated for a glucagonoma with multiple liver metastases, migratory necrolytic erythema, dilated cardiomypathy and diabetes that dramatically improved after a dacarbazin-based chemotherapy, allowing subsequent surgical resection of the primary. " 01/01/2000 - "[Chemoembolization followed by systemic chemotherapy with dacarbazine for a metastatic glucagonoma]."
2.	Pancreaticoduodenectomy 01/01/2008 - "A case of glucagonoma at the uncinate process of the pancreas successfully treated by pancreaticoduodenectomy." 09/01/2012 - "Diabetes Cured by Pancreaticoduodenectomy: A Case Report of Glucagonoma Masquerading as Carcinoma of the Head of Pancreas." 12/04/1998 - "After a pylorus-preserving partial duodenopancreatectomy with lymph node dissection N1/N2, histology confirmed a gastrinoma of the duodenum and a glucagonoma of the pancreas (pT3pN1pMx). " 09/01/2012 - "An antral gastrectomy, total pancreatoduodenectomy, colecistectomy and truncal vagotomy was performed and histopathologic examination revealed a combination of neuroendocrine tumors: gastrinomas, somastotinomas, glucagonomas and insulinomas. "
3.	Injections 10/01/2010 - "In conclusion, subcutaneous octreotide injections were beneficial in this dog with glucagonoma-associated NME. " 02/01/2014 - "Glucagonoma with necrolytic migratory erythema exhibiting responsiveness to subcutaneous octreotide injections."
4.	Subcutaneous Injections 05/29/1987 - "A newly developed, long-acting somatostatin analogue, SMS 201-995, which the patient can self-administer as a subcutaneous injection, has proven effective in suppressing glucagon secretion from glucagonomas and, in some cases, causing remission of clinical symptoms."
5.	Pancreatectomy 01/01/2014 - "We report a case of a meso-pancreatectomy performed on a pancreatic glucagonoma in a 58 years-old woman. " 03/01/2005 - "[Median pancreatectomy for early glucagonoma]." 05/01/1983 - "[Malignant pancreatic glucagonoma treated by total radical pancreatectomy. " 01/01/2003 - "Glucagonoma of the pancreas was diagnosed, and distal pancreatectomy with splenectomy was performed. " 09/01/1998 - "This study retrospectively reviews the patients who underwent surgery for these tumors at The Johns Hopkins Hospital from 1949 to 1996, inclusive There were 125 patients (65 males and 60 females) whose mean age was 51 +/- 1 years Fifty-eight patients (48%) had nonfunctional tumors, whereas 64 (52%) had functional tumors 35 (55%) insulinomas, 23 (36%) gastrmomas, three (5%) VIP-omas, two (3 %) glucagonomas, and one (1%) ACTHorna All patients with functional tumors presented with appropriate signs and symptoms of hormonal excess, 86% of patients with nonfunctional tumors presented with weight loss, abdominal pain, or jaundice Preoperaüve computed tomography (CT) correctly localized the tumor in 66 (76%) of 87 patients, angiography in 45 (58%) of 78 patients, and CT plus angiography in 54 (79%) of 68 patients Tumors were benign in 60 patients (48%), malignant m 65 patients (52%), and were located in the head, neck, or uncinate process of the pancreas in 54, body in 14, tail in 18, and duodenum in eight The most common operative procedures performed were 50 pancreaacoduodenectomies (40%), 39 distal pancreatectomies (31%), and 21 tumor enucleations (17%) Nine synchronous hepatic resections were performed for métastases Of the evaluable patients, 46 (43%) had postoperative complications, the most common of which were pancreatic fistula (16%), wound infection (15%), and delayed gastric emptying (8%) There were three in-hospital deaths (2 8%) With a mean follow-up of 55 +/-6 months, there have been 30 additional deaths, 23 of which were related to disease progression The overall 2-, 5-, and 10-year actuarial survival rates were 82%, 65%, and 47%, respectively The 5-year survival for patients with functional tumors was 77% compared to 52% for those with nonfunctional tumors (P = 0 025), the 5-year survival for patients with benign tumors was 91% compared to 49% for those with malignant tumors (P = 0 0004) By univanate analysis the most powerful predictor of poor outcome for patients with malignant tumors (n = 60) was positive surgiad margins (P = 0 006) This single-institution experience documents low mortality and moderate morbidity for patients treated operatively for pancreatic and penpancreaac neuroendocrine tumors The most favorable outcomes are observed in patients with benign functional tumors and in those with completely resected malignant tumors."