Metastatic glucagonoma.

We report a case of a very rare endocrine tumor of the pancreas. The patient is a 53-year-old female who has necrotizing and crusty maculopapular lesions on the upper and lower extremities and was diagnosed with bullous pemphigoid. The patient's symptoms included fatique and weight loss, and examination revealed pale sclera, angular chelitis and glossitis. Because the CA 19-9 and glucagon levels were high, and abdominal dynamic CT showed a mass in the pancreas body and metastatic lesions in the liver, the decision was made to operate. A distal pancreatectomy, splenectomy, cholecystectomy and right trisegmentectomy were performed. The histopathology of the tumor was reported as a neuroendocrine tumor, which was concordant with glucagonoma. The patient died on postoperative day 12 due to liver failure. According to the literature, when safe conditions were obtained, aggressive surgery followed by chemoterapy can increase the survival rate of these patients.