Synapse - Early-onset pancreatic neuroendocrine tumors: Clinical presentation, pathology features, and oncological outcomes

Early-onset pancreatic neuroendocrine tumors: Clinical presentation, pathology features, and oncological outcomes Journal Article

Authors:	Pulvirenti, A.; Hauser, H. F.; Fiedler, L. M.; McIntyre, C. A.; Le, T.; Reidy-Lagunes, D. L.; Soares, K. C.; Balachandran, V. P.; Kingham, T. P.; D'Angelica, M. I.; Drebin, J. A.; Jarnagin, W. R.; Raj, N.; Wei, A. C.
Article Title:	Early-onset pancreatic neuroendocrine tumors: Clinical presentation, pathology features, and oncological outcomes
Abstract:	Background: Early-Onset (EO) pancreatic neuroendocrine tumor (PanNET) is a rare disease, but whether it is clinically different from late-onset (LO) PanNET is unknown. Our study aimed to evaluate clinical differences and disease outcomes between EO-PanNET and LO-PanNET and to compare sporadic EO-PanNET with those with a hereditary syndrome. Methods: Patients with localized PanNET who underwent pancreatectomy at Memorial Sloan Kettering between 2000 and 2017 were identified. Those with metastatic disease and poorly differentiated tumors were excluded. EO-PanNET was defined as <50 and LO-PanNET >50 years of age at the time of diagnosis. Family history and clinical and pathology characteristics were recorded. Results: Overall 383 patients were included, 107 (27.9%) with EO-PanNET. Compared with LO-PanNET, EO-PanNET were more likely to have a hereditary syndrome (2.2% vs. 16%, P<0.001) but had similar pathology features such as tumor grade (P=0.6), size (2.2 Vs. 2.3 cm, P=0.5) and stageof disease (P=0.8). Among patients with EO-PanNET, those with hereditary syndrome had more frequently a multifocal disease (65% vs. 3.3%, P<0.001). With a median follow-up of 70 months (range 0-238), the 5-year cumulative incidence of recurrence after curative surgery was 19% (95% CI 12%-28%) and 17% (95% CI 13%-23%), in EO-PanNET and LO-PanNET (P=0.3). Five-year disease-specific survival was 99% (95% CI 98%-100%) with no difference with respect to PanNET onset time (P=0.26). Conclusions: In this surgical cohort, we found that EO-PanNET is associated with hereditary syndromes but has pathologic characteristics and oncological outcomes similar to LO-PanNET. These findings suggest that patients with EO-PanNET can be managed similarly to those with LO-PanNET. © 2024 Lippincott Williams and Wilkins. All rights reserved.
Keywords:	retrospective studies; pancreatic neoplasms; incidence; retrospective study; neuroendocrine tumor; pancreas tumor; pancreatectomy; neuroendocrine tumors; net; pancreas surgery; pancreatic neuroendocrine tumor; humans; human; pannet
Journal Title:	Annals of Surgery
Volume:	279
Issue:	1
ISSN:	0003-4932
Publisher:	Lippincott Williams & Wilkins
Date Published:	2024-01-01
Start Page:	125
End Page:	131
Language:	English
DOI:	10.1097/sla.0000000000005941
PUBMED:	37325926
PROVIDER:	scopus
PMCID:	PMC10724378
DOI/URL:	https://www.scopus.com/inward/record.uri?eid=2-s2.0-85180355201&doi=10.1097%2fSLA.0000000000005941&partnerID=40&md5=345db6bb4b72079954679acde98b4957
Notes:	The MSK Cancer Center Support Grant (P30 CA008748) is acknowledged in the PDF -- Corresponding author is MSK author: Alice C. Wei -- Source: Scopus