Advanced malignant insulinoma. Everolimus response and toxicity.

A 56-year-old female patient with an unremarkable history was admitted to hospital in August 2009 for constitutional symptoms with a weight loss of 8 kg over the previous year, early satiety, and epigastric pain treated with omeprazole and domperidone on an outpatient basis. Physical examination revealed hepatomegaly extending 3 cm below the right costal margin and palpation of the spleen apex in the left hypochondriac region. Laboratory test results included: Hb, 11.3 g/dL (<12 g/dL); ESR, 73 mm/h (>20 mm/h); HbA1c, 6.7% (>5.7%); basal blood glucose, 117 mg/dL (60--100 mg/dL); LDH, 708 (>380 U/L). Fiberoptic gastroscopy showed a 3 cm × 4 cm lesion extrinsically compressing the antrum. An abdominal CT scan revealed multiple focal hepatic lesions with characteristics of hypervascular metastasis affecting 70% of the liver, and a focal lesion in the tail of the pancreas that caused splenic vein thrombosis. Ultrasound-guided FNA of the liver disclosed hepatic metastases from a well differentiated, synaptophysin-positive neuroendocrine carcinoma. Ki67 and chromogranin A could not be tested in the sample. Octreoscan showed a positive activity of somatostatin receptors in the liver. Tissue from primary tumor could not be obtained, and a histological characterization was therefore not possible. Based on a diagnosis of metastatic, well differentiated neuroendocrine tumor (NET), chemotherapy was started with streptozocin and adriamycin. Two days later, the patient was admitted to hospital for urinary tract infection and pain in the liver, and required dexamethasone. She had hyperglycemic decompensation, which was treated with subcutaneous insulin as a basal-bolus regimen, which was continued after discharge. A CT scan performed in April 2010 showed a significant decrease in the size and number of liver metastases, and a slight decrease in size of the pancreatic