Neuroendocrine tumors (NETs) includes a broad family of tumors, the most frequent ones are in the gastrointestinal tract, lung, thymus, and pancreas. Gas-trointestinal and Pulmonary NETs may present with symptoms attributable to hormonal hypersecretion, which include intermit-tent flushing and diarrhea, or symptoms related to Cushing Syndrome. The variability of clinical manifestation may lead to late referral or misdiagnosis. In this retrospective study we analyzed 27 patients with NET of various primary tumor sites treated at our department during 20062018. Tumors were graded and staged according to histopathological criteria and their extent of metastases. Hormonal analyses have been done at each presentation. Immunohistochemical (IHC) staining for specific hormonal markers was executed in all tumors. 20 patients (12 women) presented with gastroentero-pancreatic tumors, 10 with pancreatic localization, 6 jejuno-ileal, 3 rectal and 1 gastric determination. 17 were referred to our department after surgery, with no hormonal work-up prior surgery, 3 had been primary endocrine investigated, all had developed symptoms a few years before diagnosis, and all presented with IHC staining for Synaptophisin and Chromogranin A, 2 of them got lost in follow-up. We investigated 7 patients with pulmonary NET, 5 of them were diagnosed primary in our department with ectopic Cushing, with a positive outcome after surgery and the other 2 were referred with atypical forms, requiring chemotherapy; 3 out of 27 patients have been lost in follow-up. The study revealed that neuroendocrine tumors often are late referred or misdiagnosed, and still a challenge, emphasizing the importance of multidisciplinary cooperation.
18 - 21 May 2019
European Society of Endocrinology