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Annals of Surgical Oncology
Published in: Annals of Surgical Oncology 12/2023

Open Access 30-08-2023 | Tuberous Sclerosis | Pancreatic Tumors

Long-Term Outcomes of Tuberous Sclerosis Complex-Associated Non-functional Pancreatic Neuroendocrine Tumors: Should We Be More Conservative?

Authors: Shahrzad Arya, MD, Marco Ventin, MD, Martina Nebbia, MD, Carlos Fernandez-del Castillo, MD, Gabriella Lionetto, MD, Motaz Qadan, MD, PhD, Keith D. Lillemoe, MD, Vikram Deshpande, MD, Onofrio A. Catalano, MD, PhD, Elizabeth A. Thiele, MD, PhD, Cristina R. Ferrone, MD

Published in: Annals of Surgical Oncology | Issue 12/2023

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Abstract

Background

Hereditary syndromes such as tuberous sclerosis complex (TSC) account for 10% of pancreatic neuroendocrine tumors (PNETs). Surgical intervention is the current standard of care for sporadic PNETs (spPNETs) that are >2 cm in size. We compared the long-term outcomes of resected TSC-PNETs with patients with spPNETs.

Methods

We conducted a retrospective review of perioperative data and outcomes of TSC-PNETs compared with spPNETs. Inclusion criteria involved selecting patients whose tumors were no larger than 5.1 cm, the maximum size observed in the TSC-PNET group.

Results

Of the 347 patients resected for PNETs, 14 were TSC-PNETs and 241 were non-functional spPNETs. The median age for the whole cohort was 56 years (interquartile range [IQR] 21.0) and 47% were female. The median follow-up was 103.8 months (95% confidence interval [CI] 89.2–118.6). Specifically, 14 patients with TSC-PNETs and 194 patients with spPNETs were included. Compared with spPNETs, patients with TSC-PNETs were operated on at a younger age (24.0 vs. 57.5 years; p < 0.001), were more frequently multifocal (28.5% vs. 0.0%; p < 0.001), were more likely to undergo minimally invasive operations (78.6% vs. 24.3%; p < 0.001), and had more R1 resections (28.6% vs. 5.7%; p = 0.006). Local and distant tumor recurrence was only observed in the spPNET group. The 5-year mortality rates for the spPNET and TSC-PNET groups were 6.2% and 0.0%, respectively. No PNET-related deaths were observed among TSC-PNETs.

Conclusion

None of the TSC-PNET patients recurred after a median follow-up of 78.0 months. The risk-benefit of aggressive pancreatic operations in TSC-PNET patients is still unclear and our findings suggest a conservative approach should be considered.
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Metadata
Title
Long-Term Outcomes of Tuberous Sclerosis Complex-Associated Non-functional Pancreatic Neuroendocrine Tumors: Should We Be More Conservative?
Authors
Shahrzad Arya, MD
Marco Ventin, MD
Martina Nebbia, MD
Carlos Fernandez-del Castillo, MD
Gabriella Lionetto, MD
Motaz Qadan, MD, PhD
Keith D. Lillemoe, MD
Vikram Deshpande, MD
Onofrio A. Catalano, MD, PhD
Elizabeth A. Thiele, MD, PhD
Cristina R. Ferrone, MD
Publication date
30-08-2023
Publisher
Springer International Publishing
Published in
Annals of Surgical Oncology / Issue 12/2023
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-023-14157-0

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