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Endocrine Abstracts (2024) 99 EP554 | DOI: 10.1530/endoabs.99.EP554

1National & Kapodistrian University of Athens, 2nd Surgical Department, Aretaieion Hospital


Background: Neuroendocrine neoplasms (NENs) are rather uncommon while they have an incidence of no more than 0,5 per 100.000 people. The aim of this study is to investigate the prevalence of second neoplasms among patients diagnosed with sporadic NENs.

Methods: Clinical and histological data from patients followed to our NENs clinic between 2020-2023 were retrospectively evaluated. Patients with a known genetic syndrome were excluded from the analysis.

Results: A total of 40 patients were evaluated, 60% of whom were women, with an age range at diagnosis of 18-78 years. In our cohort, 17 patients were diagnosed with pancreatic NEN (42,5%), 7 patients (17,5%) with small bowel NEN, 7 patients (17,5%) with gastric NEN, 3 patients (7,5%) with appendix NEN, 3 patients (7,5%) with rectal NEN, 2 patients (5%) with lung NEN and one (2,5%) with duodenal NEN. Among these tumors 7 (17,5%) were functioning (three with carcinoid syndrome, two with insulinoma, one with VIPoma, one with gastrinoma). Regarding their differentiation, 29% of the gastroenteropancreatic NENs had a Ki-67 index below 3%, being grade 1, 66% had a Ki-index between 3-20%, being grade 2, one patient had neuroendocrine carcinoma and in one patient no Ki-67 was available. Twenty-eight seconds neoplasms were documented in 22 (55%) of the patients; 5 patients had more than one second neoplasms. The most common diagnoses were adrenal incidentalomas (9, 32%), followed by meningiomas (3, 11%) and 2 (7%) for each of papillary thyroid carcinoma, pancreatic adenocarcinoma, and prostate cancer. From the rest of second neoplasms 3 were malignant and 7 benign neoplasms. Regarding the time of presentation, 10 (36%) had a synchronous diagnosis together with the NEN, 6 of them had a metachronous diagnosis of the secondary neoplasm, while in 12 (43%) patients the NEN diagnosis followed the other neoplasm. Overall, one fourth of the second neoplasms were malignant.

Conclusions: In our study, more than half of the patients diagnosed with NEN displayed in parallel a second neoplasm. This observation is suggestive of a possible genetic association triggering these neoplasms. Further prospective clinical and genetic studies will be needed to elucidate these mechanisms.

Volume 99

26th European Congress of Endocrinology

Stockholm, Sweden
11 May 2024 - 14 May 2024

European Society of Endocrinology 

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