Glucagonoma: “A Diagnostic Challenge”
Abstract
Introduction: Glucagonoma is a rare pancreatic α-islet cell tumor accounting for nearly 1% of all the neuroendocrine tumors (NET), with an incidence of 1 in 20 million people. We present here a patient with glucagonoma with uncontrolled diabetes mellitus (DM) for over ten years and necrolytic migratory erythema (NME) for two years. Clinical Case: A 50-year-old Caucasian male was referred to the endocrinology team for uncontrolled DM type 2. The DM was diagnosed over 10 years prior with initial HbA1c of 10.2% and remained uncontrolled with HbA1c >9%, despite compliance with lifestyle modifications and treatment with multiple agents including metformin, glipizide, empagliflozin, and pioglitazone. Given poor glycemic control, the treatment was escalated to glargine and lixisenatide. Over the years, the patient presented with unintentional weight loss of 105 pounds, fatigue, normocytic normochromic anemia, and diffuse erythematous rash with plaques and scaling on the trunk, flexor surfaces of lower and upper extremities, soles of hands and feet. Punch biopsy of the lesions revealed neutrophilic infiltration. He denied diarrhea, deep venous thrombosis, pulmonary embolism, psychiatric symptoms, or family history of neuroendocrine tumor (NET) or MEN 1 syndrome. A CT chest abdomen and pelvis revealed a 5 cm mass on the pancreatic tail along with liver metastasis. The biopsy confirmed a well-differentiated NET. Serum glucagon and chromogranin-A levels were elevated at 2967 pg/mL (N:…
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