Cases reported "Glucagonoma"

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1/8. Cystic glucagonoma: A rare variant of an uncommon neuroendocrine pancreas tumor.

    Glucagon-producing neuroendocrine tumors typically present with a characteristic constellation of symptoms including necrolytic migratory erythema, non-insulin-dependent diabetes, weight loss, anemia, glossitis, and an increased thrombotic tendency. Most glucagonomas are solid and arise in the body or tail of the pancreas. We report two cases of cystic glucagonoma, one found incidentally in an asymptomatic patient and one in a patient with weight loss and diabetes but no rash. In the first patient, distal pancreatectomy and splenectomy were curative, whereas the second patient continued to exhibit elevated serum glucagon levels and symptoms of glucose intolerance in the absence of demonstrable metastases. Cystic glucagonoma is a unique variant of classic glucagonoma and should be considered in the differential diagnosis of cystic pancreatic neoplasms.
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2/8. glucagonoma syndrome: a case report.

    glucagonoma is a very rare islet cell tumor of the pancreas. We present a case of pancreatic tail tumor with the typical glucagonoma syndrome of necrolytic migratory erythema (NME), diabetes mellitus (DM), anemia, weight loss and glossitis. After complete resection of the pancreatic tumor, the glucagonoma syndrome subsided. In reviewing 120 cases of glucagonoma in the literature, the average tumor diameter was 3.6 cm. Most (68.1%) of the tumors occurred in the pancreatic tail. Two-thirds of the reported glucagonomas were malignant and 53.5% metastasized to other organs. The curative resection rate was 45.8%. A triad of pancreatic tumor, NME and DM should lead to the diagnosis of glucagonoma.
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3/8. dermatitis, glossitis, stomatitis, cheilitis, anemia and weight loss: a classic presentation of pancreatic glucagonoma.

    Glucagonomas are rare tumors. They are predominantly located in the body or tail of the pancreas and display a constellation of signs and symptoms referred to as glucagonoma syndrome. The term necrolytic migratory erythema is used to characterize the distinctive rash associated with this syndrome. This report describes a classic presentation consisting of dermatitis, glossitis, stomatitis, angular cheilitis, anemia, and weight loss that was associated with the finding of a pancreatic mass and a markedly elevated plasma glucagon level. After pancreatic resection, the patient had complete resolution of the rash and normalization of plasma glucagon.
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4/8. Clinical experience in diagnosis and treatment of glucagonoma syndrome.

    BACKGROUND: Pancreatic endocrine tumors are uncommon neoplasms and can lead to systemic disorder including glucagonoma syndrome, a very rare prototypical paraneoplastic phenomenon. The aim of this study was to assess the diagnosis and surgical strategy for the treatment of glucagonoma syndrome. methods: The clinical data of a case of pancreatic head tumor with typical glucagonoma syndrome of necrolytic migratory erythema (NME), diabetes mellitus (DM), anemia, and glossitis were retrospectively analyzed. RESULTS: Cutaneous eruption occurred mainly in the groin, extremities, thighs, buttocks, and perineum. A highly elevated level of serum glucagon was detected by radioimmunoassay. A tumor located in the head of the pancreas was well-defined by pre and intra-operative ultrasonography, contrast enhanced computed tomography, and magnetic resonance imaging. Tumor enucleation was performed, showing significantly improved symptoms. Near complete resolution of NME was shown one week after surgery. Surgical complications or recurrence was not found. CONCLUSIONS: The diagnosis of glucagonoma syndrome is established by marked clinical features such as NME as the hallmark clinical finding, hyperglucagonemia, and radiographically demonstrated neuroendocrine tumor. The topographic diagnosis of glucagonoma can be achieved by combined imaging methods. Enucleation of tumor is a valuable treatment for solitary pancreatic tumor without peripancreatic invasion, liver metastasis, and pancreatic duct compression.
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5/8. Diagnostic challenge of glucagonoma: case report and literature review.

    OBJECTIVE: To report the diagnostic difficulties encountered in a case of glucagonoma. methods: We provide a literature review and present the clinical findings, pertinent laboratory data, and results of related studies in a patient with a glucagonoma. RESULTS: A 54-year-old-man, with no relevant history of endocrine disorders, presented to the hospital with a 5-year history of recurrent stomatitis and glossitis, a more recent weight loss of 11.5 kg, and recurrent pruritic maculae on the scalp in conjunction with raised erythematous maculae in the scrotal region and perineum that gradually migrated to the distal extremities, becoming bullous and painful. The patient was hospitalized, and because of the dermatologic findings suggestive of necrolytic migratory erythema, the presence of a glucagonoma was suspected. His blood glucose levels were in the normal range. Glucagon levels were found to be elevated, and imaging studies confirmed the presence of an enlarged mass in the pancreatic tail, without evidence of extension to surrounding structures. liver metastatic lesions were also excluded. After surgical removal of the tumor, the skin and oral mucosal lesions disappeared spontaneously. The histologic appearance and immunohistochemical staining results confirmed the diagnosis of a glucagonoma. Subsequently, all related symptoms resolved, and the glucagon levels normalized. CONCLUSION: The diagnosis of glucagonoma is often delayed. Clinicians should be aware of the unusual initial manifestations of this tumor and the potential for less than a full spectrum of the characteristic features of the glucagonoma syndrome.
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6/8. A functional study of a case of glucagonoma exhibiting typical glucagonoma syndrome.

    A 46-year-old man had a 7-year history of severe rash, which was then diagnosed as necrolytic migratory erythema. He had a weight loss of 6 kg, abnormal glucose tolerance test findings, anemia, glossitis, hair loss, and hypoproteinemia. plasma amino acids levels were significantly decreased, and the fasting plasma glucagon (IRG) level was high at 5000 to 8000 pg/ml. Circulating IRG significantly increased after oral glucose loading, meal ingestion, and arginine infusion, and decreased with somatostatin infusion and insulin-induced hypoglycemia. No other gut or pancreatic hormone levels in plasma were elevated. plasma IRG was eluted by gel-filtration, mainly in the position of true glucagon (MW 3500) by antiserum 30K. The rash was markedly improved after infusion of amino acids. Computerized tomography (CT) scan and celiac angiography revealed a large pancreatic tumor with multiple liver and lymph node metastases. The pancreatic tumor was totally resected, and was identified as glucagonoma by immunohistochemical technique. Since the plasma IRG levels remained high after surgery, the patient received dimethyltriazenoimidazole carboxamide therapy. After several courses of this treatment, plasma IRG levels decreased to 1000 to 2000 pg/ml, and the hepatic metastases were remarkably diminished in size.
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7/8. glucagonoma syndrome: case report and literature review.

    The glucagonoma syndrome is characterized by dermatitis, glucose intolerance, hypoaminoacidemia, and hyperglucagonemia secondary to an alpha-cell tumor of the pancreas. Other clinical features include anemia, glossitis, and weight loss. A 62-year-old woman with the syndrome sought medical attention for a chronic dermatitis. A skin biopsy was suggestive of necrolytic migratory erythema. A glucagonoma was surgically removed from the tail of the pancreas. review of the literature indicates that 56 proven and 33 probable cases of glucagonoma syndrome have been reported.
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8/8. Rapid resolution of necrolytic migratory erythema after glucagonoma resection.

    A 55-year-old man presented with an 11-year history of necrolytic migratory erythema and glossitis. After the patient's serum glucagon was demonstrated to be elevated, computed tomography scan revealed a mass involving the head of the pancreas. The patient underwent a Whipple-type pancreatico-duodenectomy and his rash resolved completely 6 days after tumor resection. He received no adjuvant treatment. A discussion of the varying theories regarding the pathogenesis and treatment of glucagon-associated necrolytic migratory erythema is presented.
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