Rationale.Pancreatic neuroendocrine tumors (NETs) are rare neoplasms that develop from the endocrine tissues of the pancreas. They have a better overall prognosis than pancreatic adenocarcinoma. However, all commonly used classification systems reflect a separation between more indolent, well-differentiated tumors and far more aggressive poorly differentiated types that behave clinically more like small-cell carcinoma of the lung.

Objective.To present the case of a 62-year-old man with an aggressive pancreatic NET, with liver, splenic and bone metastases who underwent multidisciplinary treatment including several lines of chemotherapy, somatostatin analogs and radiotherapy.

Methods and Results.The patient is a smoker and an occasional drinker, known with type two diabetes mellitus (DM), receiving insulin therapy. He was diagnosed by contrast-enhanced computed tomography (CT) in January 2015 with a locally invasive pancreatic body mass, intraabdominal adenopathies and liver nodules, suggestive of metastases. Histopathological diagnosis was obtained through liver biopsy: neuroendocrine tumor with a 10-15% Ki67 proliferation index. Palliative chemotherapy with oxaliplatin and capecitabine was started in March 2015. In June 2015, Sandostatin LAR was added. In March 2016, he had progressive disease. Subsequently, in September 2016, bone metastasis was found within the T10 vertebra. He underwent radiotherapy for multiple bone metastases in February 2017. Progressive disease was again found during a CT examination in May 2017. His performance status has gradually worsened since then and he died in July 2017.

Discussion.As a group, well-differentiated gastroenteropancreatic NETs are generally indolent malignancies with prolonged natural history. Intermediate-grade NETs have a slightly worse prognosis than low-grade tumors.

Abbreviations: NETs – neuroendocrine tumors, NEC – neuroendocrine carcinoma, CT – computed tomography, MRI – magnetic resonance imaging, DM – diabetes mellitus, WHO – World Health Organisation, HCV – hepatitis C virus, CEA – carcinoembryonic antigen, AFP – alpha-fetoprotein, 5-HIAA – 5-Hydroxyindoleacetic acid, IHC – immunohistochemistry, EUS – endoscopic ultrasonography, EUS FNA – endoscopic ultrasonography with fine needle aspiration, CgA – chromogranin A, PRRT – peptide receptor radioligand therapy