Early-Onset Pancreatic Neuroendocrine Tumors

作者：Alessandra Pulvirenti, Haley Hauser, Laura Fiedler, Caitlin A. McIntyre, Tiffany Le, Diane 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 · 发表于：Annals of Surgery · 年份：2023 · DOI：10.1097/sla.0000000000005941 · 被引用次数：6 · 研究领域：Neuroendocrine Tumor Research Advances、Pancreatic and Hepatic Oncology Research、Lung Cancer Research Studies

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...