TY - JOUR
T1 - Early-Onset Pancreatic Neuroendocrine Tumors
T2 - Clinical Presentation, Pathology Features, and Oncological Outcomes
AU - Pulvirenti, Alessandra
AU - Hauser, Haley F.
AU - Fiedler, Laura M.
AU - McIntyre, Caitlin A.
AU - Le, Tiffany
AU - Reidy-Lagunes, Diane L.
AU - Soares, Kevin C.
AU - Balachandran, Vinod P.
AU - Kingham, T. Peter
AU - D'Angelica, Michael I.
AU - Drebin, Jeffrey A.
AU - Jarnagin, William R.
AU - Raj, Nitya
AU - Wei, Alice C.
N1 - Publisher Copyright:
© 2024 Lippincott Williams and Wilkins. All rights reserved.
PY - 2024/1/1
Y1 - 2024/1/1
N2 - 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.
AB - 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.
KW - NET
KW - neuroendocrine tumor
KW - pancreas surgery
KW - pancreatic neuroendocrine tumor
KW - PanNET
UR - http://www.scopus.com/inward/record.url?scp=85180355201&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85180355201&partnerID=8YFLogxK
U2 - 10.1097/SLA.0000000000005941
DO - 10.1097/SLA.0000000000005941
M3 - Article
C2 - 37325926
AN - SCOPUS:85180355201
SN - 0003-4932
VL - 279
SP - 125
EP - 131
JO - Annals of surgery
JF - Annals of surgery
IS - 1
ER -