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Endocrine-related Cancer May 2024Insulinoma and glucagonoma are two rare functioning neoplasms of the neuroendocrine cells of the pancreas, respectively, characterized by an uncontrolled over-secretion... (Review)
Review
Insulinoma and glucagonoma are two rare functioning neoplasms of the neuroendocrine cells of the pancreas, respectively, characterized by an uncontrolled over-secretion of insulin or glucagon, responsible for the development of the hypoglycemic syndrome and the glucagonoma syndrome. They prevalently arise as sporadic tumors; only about 10% of cases develop in the context of rare inherited tumor syndromes, such as multiple endocrine neoplasia type 1 (MEN1), neurofibromatosis type 1 (NF1), and tuberous sclerosis complex (TSC), being the result of an autosomal-dominant germline heterozygous loss-of-function mutation in a tumor-suppressor gene. Here, we reviewed the main epidemiological and clinical aspects of insulinoma and glucagonoma in the context of genetic syndromes.
Topics: Humans; Insulinoma; Glucagonoma; Pancreatic Neoplasms; Multiple Endocrine Neoplasia Type 1; Pancreas
PubMed: 38552306
DOI: 10.1530/ERC-23-0245 -
Journal of the European Academy of... Dec 2016Glucagonoma syndrome is defined by the presence of an alpha-cell secreting tumour of the pancreas, elevated levels of glucagon, and a characteristic rash called... (Review)
Review
Glucagonoma syndrome is defined by the presence of an alpha-cell secreting tumour of the pancreas, elevated levels of glucagon, and a characteristic rash called necrolytic migratory erythema (NME). NME is usually a specific and often initial finding of glucagonoma syndrome, but it may occur in other settings unassociated with an alpha-cell pancreatic tumour (pseudoglucagonoma syndrome). Glucagonoma syndrome must be distinguished from pseudoglucagonoma syndrome. Prompt recognition of NME and subsequent workup for a glucagonoma can allow for an earlier diagnosis and enhance the chances of a favourable outcome. In particular, metastases occur late, so early recognition of glucagonoma syndrome before liver metastases can be life-saving. Surgical resection is the definitive treatment for glucagonoma syndrome, although chemotherapeutic agents, somatostatin analogues and radionuclide therapy are also employed. Herein, we offer an approach to workup after identifying NME and an update on its current treatment modalities.
Topics: Glucagonoma; Humans
PubMed: 27422767
DOI: 10.1111/jdv.13752 -
In Vivo (Athens, Greece) 2023Pancreatic neuroendocrine tumors (PNETs) are pancreatic neoplasms with neuroendocrine features, divided into functioning and non-functioning. The non-functioning PNETs... (Review)
Review
BACKGROUND/AIM
Pancreatic neuroendocrine tumors (PNETs) are pancreatic neoplasms with neuroendocrine features, divided into functioning and non-functioning. The non-functioning PNETs are the largest group, and their morbidity is the result of their potential to invade surrounding tissues and metastasize. The functioning PNETs produce hormonal symptoms due to over-secretion of specific hormones. They constitute 1% to 2% of all pancreatic tumors. The use of novel imaging methods has rendered their detection more frequent. Insulinoma, the most common functioning PNET, comprises 35-40% of all functioning PNETs. Its clinical presentation is due to hyperinsulinemia and the subsequent hypoglycemia. Glucagonoma accounts for 5% of all PNETs and is the fourth most frequent functioning PNET, following insulinoma, gastrinoma, and vipoma. Its symptoms are due to the massive secretion of glucagon and ensuing hyperglycemia. The co-existence of two PNETs is a very rare entity. This report aimed to describe cases of concomitant insulinomas and glucagonomas.
MATERIALS AND METHODS
A review of the literature was performed using the PubMed database and Cochrane library aiming to identify reported cases of concomitant pancreatic insulinoma and glucagonoma. Specifically, the research was conducted using the keywords, separately and in various combination, including insulinoma, glucagonoma, cystic, pancreatic neuroendocrine tumors and hypoglycemia. Only publications in English were included in the present study.
RESULTS
A total of 8 cases of concomitant pancreatic insulinoma and glucagonoma were identified, corresponding to the period 1992-2021.
CONCLUSION
Concomitant insulinoma and glucagonoma are rare and challenging. A multidisciplinary approach is necessary for diagnosis, prognosis, and therapy.
Topics: Humans; Insulinoma; Glucagonoma; Neuroendocrine Tumors; Pancreatic Neoplasms; Hypoglycemia; Neuroectodermal Tumors, Primitive
PubMed: 37905620
DOI: 10.21873/invivo.13345 -
The American Journal of Medicine May 1987The glucagonoma syndrome is characterized by a necrolytic migratory erythematous rash, angular stomatitis, painful glossitis, a normochromic normocytic anemia, mild... (Review)
Review
The glucagonoma syndrome is characterized by a necrolytic migratory erythematous rash, angular stomatitis, painful glossitis, a normochromic normocytic anemia, mild diabetes mellitus, weight loss, a tendency to thrombosis, and neuropsychiatric disturbances. The diagnosis is made by finding a high plasma glucagon concentration in the absence of any other cause, such as renal failure or severe stress. A pancreatic alpha-cell tumor can be identified and stained by immunocytochemistry with glucagon antibodies. Optimal treatment is surgical removal, but approximately 50 percent of the tumors have metastasized by the time of diagnosis. Since the tumor is slow-growing, remission can be obtained by hepatic artery embolization to shrink hepatic secondaries or by shrinkage, in about 10 percent of patients, with the combination chemotherapeutic regimen of 5-fluorouracil and streptozotocin. The rash frequently responds to administration of zinc, a high-protein diet, and control of the diabetes with insulin. Alongside the alpha cell in the islets of Langerhans is the D-cell, which produces somatostatin and may well act physiologically as a paracrine inhibitor of glucagon release. 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.
Topics: Adenoma, Islet Cell; Erythema; Female; Glucagon; Glucagonoma; Humans; Male; Middle Aged; Octreotide; Pancreatic Neoplasms; Somatostatin; Syndrome
PubMed: 2884877
DOI: 10.1016/0002-9343(87)90424-4 -
Medicina Clinica Nov 2020
Topics: Erythema; Glucagonoma; Humans; Necrolytic Migratory Erythema; Necrosis; Pancreatic Neoplasms
PubMed: 31515063
DOI: 10.1016/j.medcli.2019.06.027 -
The New England Journal of Medicine Mar 2017
Topics: Aged; Exanthema; Glucagonoma; Humans; Male; Pancreatic Neoplasms
PubMed: 28273025
DOI: 10.1056/NEJMicm1603135 -
Nihon Rinsho. Japanese Journal of... Mar 2011
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Clinics in Dermatology 1993
Review
Topics: Glucagonoma; Humans; Pancreatic Neoplasms; Paraneoplastic Syndromes; Skin Diseases
PubMed: 8339206
DOI: 10.1016/0738-081x(93)90103-j -
International Journal of Dermatology Feb 1997
Review
Topics: Adolescent; Adult; Aged; Diagnosis, Differential; Female; Glucagonoma; Humans; Male; Middle Aged; Pancreatic Neoplasms; Prognosis; Syndrome
PubMed: 9109001
DOI: 10.1046/j.1365-4362.1997.00042.x -
Current Problems in Cancer Dec 1981
Review
Topics: Adenoma, Islet Cell; Adolescent; Adult; Aged; Child; Diagnosis, Differential; Erythema; Female; Glucagon; Glucagonoma; Glucose Tolerance Test; Humans; Male; Middle Aged; Necrosis; Pancreatic Diseases; Pancreatic Neoplasms; Prognosis; Skin; Streptozocin; Syndrome
PubMed: 6276079
DOI: 10.1016/s0147-0272(81)80014-1