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WHO Classification of Tumours
Glucagonoma, malignant
Pancreas


Definition

Glucagonoma is a glucagon-producing endocrine neoplasm arising from the alpha cells of the pancreas. It often displays vascular invasion and may metastasize to other anatomic sites
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Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



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It may be clinically associated with necrolytic erythema migrans, diarrhea, diabetes, glossitis, weight loss, malabsorption, and anemia. Almost 25% of patients experience thromboembolic complications, and 50% have liver metastases at the time of diagnosis.
Glucagonomas are occasionally part of MEN1

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Stacpoole PW, Jaspan J, Kasselberg AG, Halter SA, Polonsky K, Gluck FW, Liljenquist JE, Rabin D (1981)
A familial glucagonoma syndrome: genetic, clinical and biochemical features.
Am J Med 70: 1017-26



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Commentary: Neuroendocrine tumours of the digestive system