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. 2023 Nov;30(12):7748-7755.
doi: 10.1245/s10434-023-14157-0. Epub 2023 Aug 30.

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

Shahrzad Arya #  1 , Marco Ventin #  1 , Martina Nebbia  1 , Carlos Fernandez-Del Castillo  1 , Gabriella Lionetto  1 , Motaz Qadan  1 , Keith D Lillemoe  1 , Vikram Deshpande  2 , Onofrio A Catalano  3 , Elizabeth A Thiele  4 , Cristina R Ferrone  5
Affiliations

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

Shahrzad Arya et al. Ann Surg Oncol. 2023 Nov.

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.

Keywords: Outcomes; PNET; Pancreatic lesion; Pancreatic neuroendocrine tumor; TSC; Tuberous sclerosis complex syndrome; mTOR inhibitor.

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Figures

Fig. 1
Fig. 1
Kaplan–Meier survival curves showing (a) overall survival and (b) disease-free survival of spPNETs and TSC-PNETs. spPNETs sporadic PNETs, TSC tuberous sclerosis complex, PNETs pancreatic neuroendocrine tumors
See this image and copyright information in PMC

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