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.

Neoplasm

An organ or organ-system abnormality that consists of uncontrolled autonomous cell-proliferation which can occur in any part of the body as a benign or malignant neoplasm (tumour).


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PMID (PMCID)
26183746
 FEMALE Middle Aged
Rapid improvement of glucagonoma-related necrolytic migratory erythema with octreotide.
Kimbara S, Fujiwara Y, Toyoda M, Chayahara N, Imamura Y, Kiyota N, Mukohara T, Fukunaga A, Oka M, Nishigori C, Minami H.
Clin J Gastroenterol. 2014;7(3):255-9.
We report a 46-year-old Japanese woman with glucagonoma who presented with mucocutaneous manifestations 1 year prior to the diagnosis of the pancreatic neoplasm with multiple liver metastases.
16362349
 FEMALE Middle Aged
[The question of surgical therapy for necrolytic migratory erythema, a cutaneous disease].
Siegel R, Linse R, Rau B.
Chirurg. 2006;77(6):535-8.
We present a case of glucagonoma syndrome in a 58-year-old woman with a history of recurrent cutaneous manifestations who was referred for surgical resection of a pancreatic neoplasm after the NME was finally diagnosed.
15313692
 MALE Middle Aged
Clinical experience in diagnosis and treatment of glucagonoma syndrome.
Zhang M, Xu X, Shen Y, Hu ZH, Wu LM, Zheng SS.
Hepatobiliary Pancreat Dis Int. 2004;3(3):473-5.
Pancreatic endocrine tumors are uncommon neoplasms and can lead to systemic disorder including glucagonoma syndrome, a very rare prototypical paraneoplastic phenomenon.
12917088
 MIXED_SAMPLE Middle Aged
Reproduction of features of the glucagonoma syndrome with continuous intravenous glucagon infusion as therapy for tumor-induced hypoglycemia.
Case CC, Vassilopoulou-Sellin R.
Endocr Pract. 2003;9(1):22-5.
Symptoms that characterize the islet cell neoplasm-related glucagonoma syndrome may develop in patients receiving an infusion of exogenous glucagon.
12414844
 MIXED_SAMPLE Adult
Ghrelin expression in islet cell tumors: augmented expression of ghrelin in a case of glucagonoma with multiple endocrine neoplasm type I.
Iwakura H, Hosoda K, Doi R, Komoto I, Nishimura H, Son C, Fujikura J, Tomita T, Takaya K, Ogawa Y, Hayashi T, Inoue G, Akamizu T, Hosoda H, Kojima M, Kangawa K, Imamura M, Nakao K.
J Clin Endocrinol Metab. 2002;87(11):4885-8.
Ghrelin expression in islet cell tumors: augmented expression of ghrelin in a case of glucagonoma with multiple endocrine neoplasm type I.
12414844
 MIXED_SAMPLE Adult
Ghrelin expression in islet cell tumors: augmented expression of ghrelin in a case of glucagonoma with multiple endocrine neoplasm type I.
Iwakura H, Hosoda K, Doi R, Komoto I, Nishimura H, Son C, Fujikura J, Tomita T, Takaya K, Ogawa Y, Hayashi T, Inoue G, Akamizu T, Hosoda H, Kojima M, Kangawa K, Imamura M, Nakao K.
J Clin Endocrinol Metab. 2002;87(11):4885-8.
Ghrelin expression was identified in a case of glucagonoma associated with multiple endocrine neoplasm type I both by Northern blot analysis using total RNA and by immunohistochemistry, although the plasma ghrelin level was not elevated.
10457311
 MALE Adult
Cystic glucagonoma: A rare variant of an uncommon neuroendocrine pancreas tumor.
Brown K, Kristopaitis T, Yong S, Chejfec G, Pickleman J.
J Gastrointest Surg. 1998;2(6):533-6.
Cystic glucagonoma is a unique variant of classic glucagonoma and should be considered in the differential diagnosis of cystic pancreatic neoplasms.
10457311
 MALE Adult
Cystic glucagonoma: A rare variant of an uncommon neuroendocrine pancreas tumor.
Brown K, Kristopaitis T, Yong S, Chejfec G, Pickleman J.
J Gastrointest Surg. 1998;2(6):533-6.
Cystic glucagonoma is a unique variant of classic glucagonoma and should be considered in the differential diagnosis of cystic pancreatic neoplasms.
9432656
 FEMALE Adult
[Neuroendocrine tumors and liver transplantation: review based on a case report].
Inturri P, Galligioni A, Halmos O, Burra P, Graziotto A, Rossaro L.
Orv Hetil. 1997;138(49):3121-4.
The primary pancreatic lesion was found 20 months later in association with recurrence of neoplasm in the graft and with a paraneoplastic syndrome peculiar of glucagonoma.
8294591
 MIXED_SAMPLE Adult
Necrolytic migratory erythema: a report of three cases.
Thorisdottir K, Camisa C, Tomecki KJ, Bergfeld WF.
J Am Acad Dermatol. 1994;30(2 Pt 2):324-9.
Necrolytic migratory erythema (NME) is a cutaneous reaction pattern with specific histopathologic features that is typically associated with a functioning pancreatic islet cell neoplasm such as a glucagonoma.
2989344
 FEMALE
Glucagonoma syndrome. A clinicopathologic, immunocytochemical, and ultrastructural study.
Vandersteen PR, Scheithauer BW.
J Am Acad Dermatol. 1985;12(6):1032-9.
Although some individuals have had no associated neoplasm, a pancreatic glucagonoma is typically found in the vast majority.
6897698
 MALE Adult
Angioplastic necrolytic migratory erythema. Unique association of necrolytic migratory erythema, extensive angioplasia, and high molecular weight glucagon-like polypeptide.
Franchimont C, Pierard GE, Luyckx AS, Gerard J, Lapiere CM.
Am J Dermatopathol. 1982;4(6):485-95.
There was no associated neoplasm such as glucagonoma.