Second neoplasms in patients with sporadic neuroendocrine neoplasms (NENs)

Konstantinos Iliakopoulos; Ariadni Spyroglou; Stefanos Xatzis; Panagiota Konstantakou; Vasiliki Michalaki; Nikolaos Memos; Konstantinos Bramis; Manousos Konstadoulakis; Krystallenia Alexandraki

doi:10.1530/endoabs.102.151

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Endocrine Abstracts (2024) 102 151 | DOI: 10.1530/endoabs.102.151

EYES2024 ESE Young Endocrinologists and Scientists (EYES) 2024 Adrenals and Neuroendocrine Tumors (17 abstracts)

Second neoplasms in patients with sporadic neuroendocrine neoplasms (NENs)

Konstantinos Iliakopoulos ¹ , Ariadni Spyroglou ¹ , Stefanos Xatzis ² , Panagiota Konstantakou ¹ , Vasiliki Michalaki ¹ , Nikolaos Memos ¹ , Konstantinos Bramis ¹ , Manousos Konstadoulakis ¹ & Krystallenia Alexandraki ¹

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¹2nd Surgical Department, Aretaieion Hospital, National & Kapodistrian University of Athens, Greece; ²ENT Department, Korgialenio-Benakio Hospital, Athens, Greece

Introduction: Neuroendocrine neoplasms (NENs) are relatively rare, with an incidence of approximately 0.5 per 100,000 people. This study aims to explore the prevalence of secondary neoplasms in patients diagnosed with sporadic NENs.

Methods: We retrospectively analyzed clinical and histological data from patients treated at our NEN clinic between 2020 and 2023. Patients with known genetic syndromes were excluded from the study.

Results: Fifty patients were evaluated, 60% of whom were women, with ages ranging from 18 to 78 years at diagnosis. Within this group, 17 patients (42.5%) had pancreatic NEN, 7 (17.5%) had small bowel NEN, 7 (17.5%) had gastric NEN, 3 (7.5%) had appendix NEN, 3 (7.5%) had rectal NEN, 2 (5%) had lung NEN, and 1 (2.5%) had duodenal NEN. Among these, 7 tumors (17.5%) were functioning: 3 with carcinoid syndrome, 2 with insulinoma, 1 with VIPoma, and 1 with gastrinoma. For differentiation, 29% of gastroenteropancreatic NENs had a Ki-67 index below 3% (grade 1), 66% had a Ki-67 index between 3-20% (grade 2), 1 patient had neuroendocrine carcinoma, and in 1 patient Ki-67 was unavailable. Twenty-eight secondary neoplasms were found in 22 patients (55%), with 5 patients having multiple secondary neoplasms. The most common were adrenal incidentalomas (9, 32%), meningiomas (3, 11%), and 2 each (7%) of papillary thyroid carcinoma, pancreatic adenocarcinoma, and prostate cancer. Additionally, 3 secondary neoplasms were malignant and 7 were benign. Regarding timing, 10 patients (36%) had synchronous diagnoses, 6 had metachronous diagnoses, and 12 (43%) had NEN diagnoses following another neoplasm. One-fourth of secondary neoplasms were malignant.

Conclusion: Over half of the patients with NENs also had a secondary neoplasm, suggesting a potential genetic link. Further clinical and genetic research is needed to understand these mechanisms.