CASE HISTORY – October 2002

Glucagonoma syndrome


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Glucagonoma syndrome is mostly associated with pancreas į-cell tumors, secreting the hormone glucagon and the other products of preproglycagon.The most characteristic feature of this syndrome is the presence of necrolytic migratory erythematous rash. The annual incidence is 1 per 20 million. Though above 70% of glycagonomas are malignant, they are slow growing. At the time of diagnosis over 50–70% glucagonomas have metastasized. We present a 47-year-old woman with erythematous rash, involving the perineum, buttocks, groen, extremities and the perioral region, characteristic of  migratory necrolytic erythema. She had mild hyperglycaemia, normochromic normocytic anaemia, severe depression, and weight loss of 20 kg. Her serum glucagon level was elevated and somatostatin receptor scintigraphy showed a pancreatic tumor with hepatic and pulmonal metastases. We diagnosed glucagonoma with metastases and started with symptomatic treatment with octreotide. After one month of therapy the rash healed, weight normalised, and the patient was in a satisfactory condition. In March 2002 the patient had palliative tissue debulking operation; she recovered and octreotide treatment is continued.