Early-Onset Pancreatic Neuroendocrine Tumors: Clinical Presentation, Pathology Features, and Oncological Outcomes

Alessandra Pulvirenti, Haley F. Hauser, Laura M. Fiedler, Caitlin A. McIntyre, Tiffany Le, Diane L. Reidy-Lagunes, Kevin C. Soares, Vinod P. Balachandran, T. Peter Kingham, Michael I. D'Angelica, Jeffrey A. Drebin, William R. Jarnagin, Nitya Raj, Alice C. Wei

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)125-131
Number of pages7
JournalAnnals of surgery
Volume279
Issue number1
DOIs
StatePublished - Jan 1 2024
Externally publishedYes

Keywords

  • NET
  • neuroendocrine tumor
  • pancreas surgery
  • pancreatic neuroendocrine tumor
  • PanNET

ASJC Scopus subject areas

  • Surgery

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