PRRT as an alternative method of treatment in patient with glucagonoma syndrome: A case report

Abstract

Introduction: Glucagonoma is a rare pancreatic neuroendocrine tumor derived from alpha-cells of the islet of Langerhans. Due to oversecretion of glucagon it is associated with a characteristic paraneoplastic phenomenon, called glucagonoma syndrome, which consists of necrolytic migratory erythema (NME), weight loss, diabetes mellitus, diarrhea, normochromic normocytic anemia, deep vein thrombosis or pulmonary embolism and neuropsychiatric disturbances. Treatment modalities include surgical removal of tumor, somatostatin analogs and peptide receptor radionuclide therapy (PRRT). Case report: We present a case of 61-year-old woman diagnosed with glucagonoma in April 2012. Initially, body-caudal pancreatomy and resection of regional lymph nodes were performed. Five months after surgery, a PET-CT scan detected pathological mass with expression of somatostatin receptors in pancreatic body and metastases to regional lymph nodes. What is more, since April 2014 the patient had complained about persistent pruritus of the entire body. At present, due to the nonsurgical pancreatic mass and metastases she is treated with somatostatin analogs and PRRT. During this therapy the pruritus had decreased and currently there is no sign of cutaneous disease. Moreover, reduction of tumor size was obtained. Conclusions: PRRT may reduce tumor size and by reducing bothersome symptoms substantially improve the quality of life in patients with SSTR-positive tumors