Normoglycemic glucagonoma syndrome associated with necrolytic migratory erythema

Abstract

Glucagonoma is an extremely rare tumor of the pancreatic alpha-cells. Its estimated annual incidence is 1 case per 20 million individuals. Necrolytic migratory erythema (NME) is the hallmark clinical sign. We report a patient with normoglycemic glucagonoma and NME.

A 44-year-old male patient presented with a 4-year history of unexplained exanthema and unintentional weight loss. Skin examination revealed a mildly pruritic rash on the trunc, the extremities and the face (fig 1). One found erythematous polycyclic migratory lesions with scaling and crusting margins and central resolution. Histological examination showed parakeratotic epidermis and upper epidermal pallor with vacuolated keratinocytes (fig 2). The glucagon level was elevated at 444,5 pg/mL (normal 5,1-62,3 pg/mL). Fasting blood sugar and HbA1c where within normal range. The insulin level was raised (112 μU/mL, normal 3,21-16,32 μU/mL). CT scan of the abdomen showed two masses in the tail of pancreas, measuring 3,8 cm and 2,6 cm in diameter and thrombosis of the left common femoral vein. In the somatostatin receptor scintigraphy, the pancreatic masses stained positive. Multiple endocrine neoplasia type 1 syndrome (MEN 1) was ruled out by genetic analysis. Surgical resection of the pancreatic tail with local lymphadenectomy and splenectomy was performed. Pathological examination confirmed two low-grade neuroendocrine neoplasms consistent with glucagonoma. Tumor cells were also found in 1 out of 19 removed lymph nodes. After surgery, the patients general condition improved rapidly and the skin lesions completely disappeared.

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