Clinical presentation and outcomes of sporadic neuroendocrine neoplasms in young adults

Malczewska-Herman A, Rinke A, Holmager P, Opalińska M, McNamara MG, Netto D, Tesselaar ME, Tsoli M, Grozinsky-Glasberg S, Bel-Ange A, Clement D, González-Devia D, Garcia-Carbonero R, Grzelak O, Davi MV, Druce MG, Milanetto AC, Modica R, Megdanova V, Martin CS, Ciobanu O, Dam G, Zatelli MC, Zanata I, Del Olmo-García M, Alexandraki KI, Pavel ME, Kos-Kudla B (2026)

Publication Type: Journal article

Publication year: 2026

Journal

Book Volume: 195

Pages Range: 111-122

Journal Issue: 1

DOI: 10.1093/ejendo/lvag100

Abstract

Objective: Most neuroendocrine neoplasms (NENs) are sporadic and occur predominantly in older patients. Early-age disease onset has been increasingly observed in NENs. We aimed to assess the clinical features and outcomes of young adults with sporadic NENs. Design: This is a retrospective study that gathered 20 institutions from 12 countries under the auspices of Women in NET (WiN) ENETS group. Methods: Young patients aged 18-40 years diagnosed with sporadic NENs (well and poorly differentiated) from 2010 to 2020 were enrolled. Patients with appendiceal and type 1 gastric NETs, hereditary syndromes, synchronous malignancy, pheochromocytoma, paraganglioma, and medullary thyroid cancer were excluded. Descriptive statistics, comparisons between groups, survival, and Cox multivariable analysis were performed. Results: Overall, 478 young patients with sporadic NENs were enrolled. Localised (62%, n = 249), well-differentiated (92%, n = 433), non-functioning tumours (81%, n = 384), and women (58%, n = 277) were more prevalent. The pancreas was the most common primary site (33%, n = 159). Metastatic disease at diagnosis was more often found in men than women (47%, n = 78 vs. 33%, n = 78, P = .004). Five-year overall survival was 82%. Overall survival was significantly longer in women than men (P = .019), as well as in rectal (P = .00245) and lung (P = .0027) primary tumours compared to pancreatic NENs. The multivariable analysis identified stage, Ki-67, morphology, and gender as independent predictors of survival. Conclusions: Young patients with NENs often present with well-differentiated and localised tumours, thereby increasing the probability of curative surgical resection. Prospective research into molecular biology is needed to identify distinct NEN features in young adults and determine risk factors to tailor effective treatment strategies.

Authors with CRIS profile

Involved external institutions

Queen Mary University of London United Kingdom (GB) Aarhus University Hospital / Aarhus Universitetshospital Denmark (DK) Università degli Studi di Ferrara Italy (IT) Hospital Universitario y Politécnico de La Fe Spain (ES) Jagiellonian University / Uniwersytet Jagielloński (UJ) Poland (PL) Hadassah Medical Center / מרכז רפואי הדסה‎‎ Israel (IL) King's College Hospital (KCH) United Kingdom (GB) Hospital Universitário Fundación Santa Fe de Bogotá Colombia (CO) Aretaeio Private Hospital Greece (GR) Copenhagen University Hospital Denmark (DK) Netherlands Cancer Institute - Antoni van Leeuwenhoek Hospital (NKI / NKI-AVL) Netherlands (NL) Università degli Studi di Napoli Federico II Italy (IT) Aleksandrovska University Hospital / университетска болница "Александровска" Bulgaria (BG) Carol Davila University of Medicine and Pharmacy / Universitatea de Medicină și Farmacie „Carol Davila” (UMF București) Romania (RO) Medical University of Silesia in Katowice / Śląski Uniwersytet Medyczny w Katowicach (SUM) Poland (PL) Universitätsklinikum Gießen und Marburg (UKGM) Germany (DE) University of Manchester United Kingdom (GB) Christie NHS Foundation Trust United Kingdom (GB) National and Kapodistrian University of Athens Greece (GR) Hospital Universitario 12 de Octubre Spain (ES) University Hospital of Verona / Azienda Ospedaliera Universitaria Integrata Verona (AOUI) Italy (IT) Saint Camillus International University of Health Sciences (UniCamillus) Italy (IT)

How to cite

APA:

Malczewska-Herman, A., Rinke, A., Holmager, P., Opalińska, M., McNamara, M.G., Netto, D.,... Kos-Kudla, B. (2026). Clinical presentation and outcomes of sporadic neuroendocrine neoplasms in young adults. European Journal of Endocrinology, 195(1), 111-122. https://doi.org/10.1093/ejendo/lvag100

MLA:

Malczewska-Herman, Anna, et al. "Clinical presentation and outcomes of sporadic neuroendocrine neoplasms in young adults." European Journal of Endocrinology 195.1 (2026): 111-122.

BibTeX: Download