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The Endocrine Pancreas
Published in George H. Gass, Harold M. Kaplan, Handbook of Endocrinology, 2020
The glucagonoma syndrome is extremely rare, with approximately 100 cases reported in the literature. The mean age at diagnosis is 55, yet many patients have a long history of symptoms that suggest the tumor’s presence for 5 to 10 years. The associated clinical features include weight loss, mild diabetes, depression, and anemia. Other conditions that are more specific for glucagonoma are painful tongue enlargement and ulcers at the angles of the mouth as well as a characteristic red, migratory, oozing rash. This rash is thought to be associated with zinc deficiency and low levels of amino acids. A less common but potentially fatal complication of glucagonoma results from blood clots forming in large veins within the body in about 30% of patients. Diagnosis is easily accomplished with modern assays, in which the upper limit of normal for glucagon levels is 20 pmol/L. The cutoff for diagnosis is generally set at 50 pmol/L or higher. Other conditions that can lead to elevated glucagon levels are renal failure, hepatic failure, prolonged hypoglycemia, and severe stress. Current treatment options include surgical removal, which is the treatment of choice when technically possible. Alternative therapies in selected cases include hepatic artery embolization and chemotherapy with either streptozotocin or octreotide, a long-acting somatostatin analog.40
Endocrine emergencies with skin manifestations
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Glucagonoma is a rare neuroendocrine tumor that originates from the α-2 cells of the pancreas. Approximately 80% of tumors occur sporadically, with the remainder associated with the MEN1 (multiple endocrine neoplasia type 1) syndrome. About 75% of glucagonomas are malignant and have metastasized by the time of diagnosis.
Nail changes in systemic diseases and drug reactions
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Glucagonoma may cause a characteristic paraneoplastic disease with weight loss, diabetes mellitus, necrolytic migratory erythema, erosive stomatitis, angular cheilitis, paronychia, and very thin and brittle nails.92,93 Granulation tissue may appear.94
Necrotising migratory erythema leading to the diagnosis of a metastatic glucagonoma without diabetes
Published in Journal of Endocrinology, Metabolism and Diabetes of South Africa, 2020
On abdominal computerised tomography (CT) scan there was a large heterogeneous mass in the liver and multiple sclerotic lesions in vertebrae, pelvis and proximal femurs in keeping with metastatic foci. Single photon emission computerised tomography (SPECT) with OctreoscanTM (octreotide uptake scan) had focal increased areas of uptake in the head of the pancreas consistent with a glucagonoma and increased uptake in the liver, as well as skeletal metastases. The immunohistochemical stains on liver biopsy showed metastatic neuroendocrine tumour cells. Confirmation using a monoclonal antibody stain specifically for glucagon confirmed a glucagonoma. Serum glucagon levels were unavailable at the laboratory.
Unremitting chronic skin lesions: a case of delayed diagnosis of glucagonoma
Published in Journal of Community Hospital Internal Medicine Perspectives, 2019
Hameem I. Kawsar, Alma Habib, Azhar Saeed, Anwaar Saeed
Necrolytic migratory erythema (NME) is a rare cutaneous paraneoplastic manifestation of glucagonoma. Glucagonoma is a tumor of alpha cells of pancreas that secret glucagon, and plays a major role in blood glucose hemostasis. Elevated glucagon level due to glucagonoma can cause a variety of clinical manifestations including weight loss, diabetes, and NME. Misdiagnosis of paraneoplastic cutaneous manifestation could delay the diagnosis of glucagonoma. Here, we present an interesting case of chronic skin rash which was treated as eczema for more than 4 years, and recently as urticarial vasculitis, which was actually NME of glucagonoma. The skin rash has resolved completely with resection of the tumor.