The
glucagon-producing pancreatic
tumors or
glucagonomas are among the rarest forms of
islet cell tumors; most are malignant and usually produce a definite clinical syndrome. Mild
diabetes mellitus,
weight loss, and
anemia usually accompany the syndrome. However, only the presence of a peculiar cutaneous
rash (
necrolytic migratory erythema) and the finding of hyperglucagonemia on assay are reliable diagnostic features of the syndrome. Selective, celiac axis arteriography is the most valuable preoperative technique for localizing these
neoplasms and their common liver
metastases. Immunohistochemical and ultrastructural examinations are particularly helpful in defining the
tumor cell nature (alpha-2 islet cell) and the
peptide content (
glucagon). When the
tumor is benign (less than 30%), complete operative removal results in lasting cure; for malignant forms, surgical
therapy is mainly palliative, and adjunctive
chemotherapy should be administered. In this report, the importance of clinical recognition and operative and chemotherapeutic responses is illustrated in two patients. In each case, the characteristic
dermatitis,
diabetes mellitus,
weight loss,
anemia, and elevated plasmatic
glucagon were present. Both patients had their
tumors localized by selective angiography and underwent operative removal of the primary pancreatic lesion. In the case of benign
glucagonoma, surgical excision was curative. In the malignant one,
cytoreductive surgery plus adjunctive
chemotherapy (dimethyltriazenomidazole-carboxamide resulted in prolonged survival and significant clinical improvement. Follow-up with serum
glucagon assay has been useful in monitoring recurrence.