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The glucagonoma syndrome is characterized by dermatitis, stomatitis, elevated serum glucagon levels, abnormal glucose tolerance, weight loss, and anemia--all in association with a glucagon-secreting alpha-cell tumor of the pancreas. A review of 21 cases showed strikingly similar features. A generalized, symmetrical dermatitis initially appeared to be asteatotic or eczematous over the perineum, buttocks, and lower extremities. Gradually, a more characteristic migratory necrolytic erythema with transient bulla formation and erosions developed in intertriginous and dependent areas. Histologically, the most specific features included necrolysis of the upper epidermis, with liquefaction necrosis of the granular cell layer and subcorneal clefting or blister formation. The dermatologist is often first to examine such patients; early recognition of this syndrome with prompt surgical removal of the primary pancreatic lesion may afford cure of the neoplasm.
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PMID:Glucagonoma syndrome. Report of two cases and literature review. 19 36

The glucagonoma syndrome occurs in some but not all patients with a benign or malignant islet cell tumor and hyperglucagonemia. Manifestations may include anemia, diabetes mellitus, pruritic skin rash, glossitis, stomatitis, weight loss, diarrhea, flexible fingernails, venous thromboses, low plasma amino acid levels, and coarse folds of the jejunum and ileum. Most patients are postmenopausal women, but men and women ages 40 to 65 have been affected. The course is variable depending upon the nature of the underlying tumor. Twenty-two cases of probable glucagonoma syndrome have been reported; twelve documented with glucagon levels. The hyperglucagonemia results from elevation of the proglucagon and true glucagon immunoreactive fractions of pancreatic glucagon. Management of the rash can be accomplished rarely with topical or systemic antibiotics or corticosteroids. If the tumor is resectable, surgery reverses the syndrome. Patients with metastatic disease have responded to streptozotocin and DTIC.
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PMID:The glucagonoma syndrome and its management. 20 9

Most, if not all, of the glucagon-producing tumours of the pancreas are malignant. For this reason an early diagnosis is essential. The glucagonoma syndrome is associated with a skin rash, stomatitis, anaemia, glucose-intolerance, hypoaminoacidaemia, weight loss, elevated sedimentation rate and hyperglucagonaemia. The more important and constant findings are the skin lesion, the low level of aminoacids in the blood and the increased glucagon concentrations. The skin lesion is not pathognomonic, but any therapy-resistant bullous dermatosis which microscopically is characterized by epidermal changes should alert the clinician to suspect a glucagonoma. The syndrome can be proved by demonstration of hyperglucagonaemia and a pancreatic tumour.
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PMID:Possible entries to the diagnosis of a glucagon-producing tumour. 22 89

A 34-year-old man presented with classic glucagonoma syndrome manifested by weight loss, dermatitis, stomatitis, anemia, and mild diabetes mellitus. The diagnosis of glucagonoma was made by light and electron microscopic demonstration of a metastatic alpha cell carcinoma in a liver biopsy specimen. Plasma glucagon concentration was abnormally high. The patient also had symptoms and signs of involvement of the central nervous system. Radionuclide and CAT scans of the brain, negative CSF cytology and myelography excluded the possibility of metastases or other space-occupying lesions. Glucagon was demonstrated in the CSF. We postulate that the neurologic symptoms were due to direct or indirect effect of this hormone on the brain. Following therapy with streptozotocin and 5-fluorouracil, the patient had a subjective and objective clinical and hormonal remission of his disease including amelioration of his neurological impairment.
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PMID:Neurologic involvement in glucagonoma syndrome: response to combination chemotherapy with 5-fluorouracil and streptozotocin. 22 32

A 66-year-old male patient with non-insulin-dependent diabetes of probably 20 years' duration presented with necrolytic migratory erythema, stomatitis, anemia and weight loss. Plasma-glucagon concentration measured with Unger's antibody 30-K was 8500 pg/ml, representing a hundredfold elevation. Two thirds consisted of high molecular glucagon fractions (10 000--40 000 Dalton). This may be an important index for detection of glucagonoma with endocrine activity. After excision of the glucagonoma the clinical syndrome was reversed and the patient recovered completely. Histological and histochemical investigation confirmed that the tumor was a glucagonoma. Despite complete removal of the tumor and a normal plasma glucagon concentration, the diabetes remained unchanged. Excessive hyperglucagonemia does not appear to play a primary role in the pathogenesis of this patient's diabetes.
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PMID:[The course of diabetes and clinical findings in glucagonoma]. 52 94

A 49-year-old woman suffered from recurrent episodes of necrolytic migratory erythema over the lower legs, lower abdomen, and buttocks for more than two years. Stomatitis, glossitis and vaginitis were the accompanying symptoms and signs during each episode. The result of skin biopsy revealed superficial necrosis in the upper half of the epidermis. Laboratory examinations revealed mild glucose intolerance and hypoaminoacidemia. Fasting plasma glucagon level measured by radioimmunoassay was 890 pg/mL. Oral glucose loading test showed a paradoxical increase in plasma glucagon level up to 1,500 pg/mL. Abdominal echo, computerized axial tomography, and celiac angiography demonstrated a hypervascular tumor, 4 cm in diameters, located at the pancreatic head. Glucagonoma syndrome was confirmed and diagnosed. The patient underwent surgical resection of the tumor mass. Necrolytic migratory erythema disappeared thereafter, and the plasma glucagon level declined to 120 pg/mL. Histologically, the tumor revealed an islet cell carcinoma composed of moderately uniform cells with a few mitosis, arranged in cords and nests. Abundant characteristic secretory granules of the pancreatic A cell were found within the tumor cells by electron microscopic examination.
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PMID:[Necrolytic migratory erythema as the first manifestation of glucagonoma]. 168 96

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.
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PMID:Glucagonoma syndrome. 288 77

The features of 41 proven or suspected cases of pancreatic glucagonoma and one possible case of renal glucagonoma have been reviewed. Glucagonoma is one form of islet cell neoplasm and involves pancreatic alpha cells. It may occur more frequently in women and is more likely to be malignant than insulinoma. Patients may present with glucose intolerance, an erythematous, eczematous dermatitis, glossitis, stomatitis, vaginitis and unexplained weight loss. Anemia, hypoproteinemia, hypoaminoacidemia and hypolipidemia may also be present. Malignant glucagonoma metastasizes frequently to liver. An evaluation for possible glucagonoma may be considered in a patient with the characteristic eczematous dermatitis, glossitis or stomatitis and glucose intolerance, an unusual or atypical history of diabetes mellitus, or hepatomegaly with other characteristics of glucagonoma. Initial evaluation may include measurement of fasting plasma glucagon concentration, and an oral glucose tolerance test with measurements of plasma glucose and glucagon levels. Extreme fasting hyperglucagonemia, and a paradoxical rise in plasma glucagon concentrations after glucose ingestion should strongly suggest the presence of glucagonoma. Radiographic demonstration of pancreatic glucagonoma is best carried out by celiac arteriography. Surgical excision of the tumor is the treatment of choice. Nonresectable lesions may respond to chemotherapy with streptozotocin. Treatment for the various dermatologic or metabolic complications of glucagonoma which include glucose intolerance, hypoproteinemia, hypocholesterolemia and anemia may not be satisfactory. Glucose intolerance is usually mild and may be adequately treated with dietary or insulin therapy. Rarely, glucagonoma with massive destruction of the pancreas or other factors may induce severe glucose intolerance. In contrast, the anemia, skin rash, and hypoproteinemia do not respond to conservative therapies tested thus far. Glucagonoma is a model for studying the importance of glucagon in causing the hyperglycemia of diabetes mellitus. Study of patients with glucagonoma does suggest that glucagon has some role in the etiology of hyperglycemia in diabetic states; however, as in studies on diabetes, investigations on glucagonoma do not demonstrate that glucagon has a primary role in producing severe glucose intolerance.
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PMID:Clinical and metabolic aspects of glucagonoma. 698 81

Glucagonoma is a rare pancreatic tumor, necrolytic migratory erythema is its distinctive feature and it is often associated with diabetes mellitus, weight loss, anemia, hypoaminoacidemia, glossitis and stomatitis. We reported a case of glucagonoma misdiagnosed as "eczema" and "benign hepatic anginoma" for 3 years. His blood glucagon level was 1,758 ng/L. The results of abdominal B-mode ultrasonography and CT scan were negative, but selected arteriogram showed a tumor mass between the pancreatic body and tail. Before operation, treatment with octreotide and supply of amino acids were given with improvement of the skin lesion. After resection of the tumor from pancreas, necrolytic migratory erythema disapeared, but his blood level of glucagon and amino acids did not improve. It is suggested that any diabetic patient with chronic skin damage should be checked for blood glucagon level. In suspected cases, selected arteriogram will be helpful for location of the tumor. Vigorous resection of the pancreatic tumor should be done as soon as possible, even though there is already metastases.
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PMID:[Report of a case of glucagonoma misdiagnosed as "eczema" and "hepatic angioma" for three years and review of literature]. 764 42

Glucagonoma syndrome is a paraneoplastic phenomenon characterized by an islet alpha-cell pancreatic tumor, necrolytic migratory erythema, diabetes mellitus, weight loss, anemia, stomatitis, thromboembolism, and gastrointestinal and neuropsychiatric disturbances. These clinical findings in association with hyperglucagonemia and demonstrable pancreatic tumor establish the diagnosis. Glucagon itself is responsible for most of the observed signs and symptoms, and its induction of hypoaminoacidemia is thought to lead to necrolytic migratory erythema. Liver disease and fatty acid and zinc deficiency states may also contribute to the pathogenesis of the eruption in some cases. Most patients are diagnosed too late in the clinical course for cure, but successful palliation of symptomatology can usually be achieved with surgical and medical intervention. This paper reviews the glucagonoma syndrome, paying particular attention to its cutaneous features, and provides new perspectives in our current understanding of this phenomenon.
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PMID:The glucagonoma syndrome: a review of its features and discussion of new perspectives. 1137 Jul 94


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