Endocrine Abstracts

Introduction: Neuroendocrine neoplasms (NENs) are rare and heterogeneous neoplasms with variable biological behaviour but generally slow progression.

Methods/design: 355 patients with NENs registered in our data-base have been studied (166 females, mean age: 52 years; range: 11–88), during the period 2004–2014. TNM system has been used for staging and proliferation index Ki-67 for grading.

Results: Out of 355 patients, 35 (10%) had neoplasms in the context of familiar disorders: MEN-1(n=32) and Von Hippel Lindau (n=2). The following primary sites were registered: stomach: 56 (15.8%); duodenum: 13 (3.7%); pancreas: 131 (36.9%); small intestine: 40 (11.3%); appendix: 31(8.8%); colon: 14 (4%); lung: 27 (7.6%); thymus: 1 (0.3%), other sites: 22 (6.2%), unknown origin: 15 (4.2%); 4 (1.2%) had two concomitant neoplasms. 54 (15.3%) had functional syndrome. Metastatic deposits were found in 103 (29%) patients: bones: 16 (4.5%), lung: 5 (1.4%), liver: 94 (22.5%), peritoneum: 7 (2%), pancreatic: 2 (0.6%), brain: 2 (0.6%), omentum: 2 (0.6%), ovaries/kidneys/spleen: 1 (0.28%). 139 (47.8%) patients had Ki-67≤2% (grade 1), 111 (31.3%) 3–20% (grade 2), 24 (8.2%) >20% (grade 3) for gastro-intestinal NENs and for lung 4 (1.4%) atypical, 10 (3.4%) atypical, 1 (0.3%) large and 1 (0.3%) small cell lung carcinomas and 1 (0.3%) atypical thymic NEN. Stage1 were found in 135 (38.8%), stage2: 63 (18.1%), stage3: 48 (13.8%), stage4: 102 (29.3%). 197 patients had surgical removal of the neoplasms, 46 had an endoscopic resection. The other systemic or local therapies that the patients received as monotherapy or in combination as first line treatment are as following: somatostatin analogs (n=100), chemotherapy (n=45), neoplasm-targeted molecular therapy (n=22), radionucleotides (n=9), conventional radiotherapy (n=6), cauterisation with radiofrequencies/RFA (n=8), chemoembolisation (n=6), interferon (n=2). 99 patients received second, 44 third, 23 forth, 12 fifth, 5 sixth line treatment. In the last follow-up of the present analysis 21 patients died from their disease: five had grade1 neoplasm, ten grade 2, two grade 3, one atypical thymic and another atypical lung NEN but two patients did not have an available Ki-67.

Conclusion: The present registry imply that the majority of the patients with NENs have slow progression and long survival despite the presence of disseminated disease, confirming the necessity of the recent introduced therapeutic and diagnostic options following the guidelines as well as their management from referral centres under multidisciplinary teams.