Glucagonoma accounts for 5% of all PNETs and is the fourth most frequent functioning PNET, following insulinoma, gastrinoma, and vipoma. (bvsalud.org)
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. (bvsalud.org)
RESULTS: A total of 8 cases of concomitant pancreatic insulinoma and glucagonoma were identified, corresponding to the period 1992-2021. (bvsalud.org)
CONCLUSION: Concomitant insulinoma and glucagonoma are rare and challenging. (bvsalud.org)
Syndrome12
The presence of glucagonoma syndrome, the symptoms that accompany the pancreatic tumor, as well as elevated levels of glucagon in the blood, are what is used to diagnose glucagonoma. (wikipedia.org)
When a person presents with a blood glucagon concentration greater than 500 mg/mL along with the glucagonoma syndrome, a diagnosis can be established. (wikipedia.org)
Malignant glucagonomas are islet cell pancreatic tumors that are discovered because of glucagonoma syndrome (in which the glucagonoma autonomously secretes glucagon), because of local mass effects, or incidentally. (medscape.com)
Glucagonomas that are not associated with glucagonoma syndrome are diagnosed in various ways. (medscape.com)
The vast majority of glucagonomas are sporadic (80%) with the remainder associated with multiple endocrine neoplasia type 1 (MEN1) , an inherited tumor predisposition syndrome, or Mahvash disease, an extremely rare cause of familial pancreatic α-cell hyperplasia and glucagonoma due to inactivating mutations in the glucagon receptor ( GCGR ) gene. (medscape.com)
Glucagonoma very rarely is part of multiple endocrine neoplasia (MEN) type 1 syndrome (also called Wermer syndrome), and in such cases, the glucagonoma appears as a single, biologically inactive lesion. (medscape.com)
The rarity of pseudoglucagonoma syndrome, or necrolytic migratory erythema occurring in the absence of a glucagonoma, warranted the discussion of this case. (cdlib.org)
Although only having diagnosed the glucagonoma syndrome (GS) once, I have always been fascinated by necrolytic migratory erythema (NME), and conditions that mimic NME, the so-called pseudoglucagonoma syndrome (PGS). (aad.org)
Necrolytic4
Typically associated with a rash called necrolytic migratory erythema, weight loss, and mild diabetes mellitus, most people with glucagonoma contract it spontaneously. (wikipedia.org)
Recurrent cutaneous manifestation of GLUCAGONOMA characterized by necrolytic polycyclic migratory lesions with scaling borders. (bvsalud.org)
Tumors3
Glucagonoma accounts for approximately 1% of neuroendocrine tumors, although this may be an underestimate given that glucagonoma is associated with non-specific symptoms. (wikipedia.org)
A glucagonoma is a rare neuroendocrine tumor that originates almost exclusively in the pancreas and probably accounts for 1% of all neuroendocrine tumors. (medscape.com)
The correct recognition of NME is very important, because it may allow early detection either of glucagonoma or of extrapancreatic, glucagon-secreting tumors. (medscape.com)
Diagnosis of glucagonoma1
It is important to note that not all cases of hyperglucagonemia will lead to a diagnosis of glucagonoma. (wikipedia.org)
Tumor5
Glucagonoma is a very rare tumor of the pancreatic alpha cells that results in the overproduction of the hormone, glucagon. (wikipedia.org)
A glucagonoma is a rare neuroendocrine tumor with nearly exclusive pancreatic localization. (medscape.com)
A glucagonoma is a pancreatic alpha-cell tumor that secretes glucagon , causing hyperglycemia and a characteristic rash. (msdmanuals.com)
A glucagonoma happens when a tumor arises among a group of glucagon-producing cells. (hopkinsmedicine.org)
These findings suggest a rare glucagonoma-like NET appendiceal tumor that had metastasized to the surface of ovary and were unresponsive to CRC chemotherapy regimens. (edu.au)
Malignant2
In 75-80% of cases, the glucagonoma starts in malignant form, and in 50% of these cases, metastasis exists at diagnosis. (medscape.com)
Malignant glucagonoma of the pancreas diagnosed through anemia and diabetes mellitus. (asean-endocrinejournal.org)
Glucagon receptor1
Additionally, those with Mahvash disease have an increased risk for glucagonoma, as the glucagon receptor gene (GCGR) is mutated. (wikipedia.org)
Cystic1
Malign cystic glucagonoma presented with diabetic ketoacidosis: Case report with an update. (asean-endocrinejournal.org)
Symptoms2
Glucagonoma-associated neuropsychiatric and affective symptoms: Diagnostic dilemmas raised by paraneoplastic phenomena. (asean-endocrinejournal.org)
Glucagonoma leads to diabetes-like symptoms and other severe symptoms, including: high blood sugar. (shakuhachi.net)
Glucose1
Weight loss, the most commonly associated effect with glucagonoma, results from the glucagon hormone, which prevents the uptake of glucose by somatic cells. (wikipedia.org)
Metastatic1
In contrast localised glucagonoma without metastatic spread may have prolonged disease free survival with radical resectional surgery. (dundee.ac.uk)
Metastases1
Cervical metastases of glucagonoma in a patient with multiple endocrine neoplasia type 1: Report of a case. (asean-endocrinejournal.org)
Erythema1
Although classically associated with a pancreatic glucagonoma, this patient experienced this figurate erythema in the setting of fatty liver disease with no glucagonoma. (cdlib.org)
Diabetes1
Diabetes is not present in all cases of glucagonoma, but does frequently result from the insulin and glucagon imbalance. (wikipedia.org)
Lesions1
NME presents in about 70% of cases of glucagonoma, and is characterized by erythematous lesions over the distal extremities and the groin area. (wikipedia.org)
Cases3
Fewer than 251 cases of glucagonoma have been described in the literature since their first description by Becker in 1942. (wikipedia.org)
The causes of sporadic glucagonomoas remain unknown, and it is noteworthy that some cases of NME without glucagonoma have been reported. (medscape.com)
However, it is noteworthy that some cases of NME without glucagonoma have been reported. (medscape.com)
Disease1
People who develop glucagonoma from Mahvash disease also do not develop NME, implying that working glucagon receptors are needed in order for NME to be present in a person. (wikipedia.org)
Cells1
Glucagonoma results from the overproduction of glucagon, a peptide hormone located in the pancreatic alpha cells. (wikipedia.org)
Patients1
Glucagonoma may also occur as a single microadenoma found incidentally at autopsy in elderly patients. (medscape.com)