Pancreatic Neuroendocrine Tumors in French VHL Mutation Carriers
Authors' conclusion
The risk of metastases associated with VHL-related PanNETs remains low (24%) but increases with tumor size greater than 28 mm, higher grade, and in case of PV, located in VHL exon 1. These data might help improve the management of these patients, who should be referred to an expert center.
Study context
- Population
- Patients with documented germline PV in the VHL gene and pancreatic neuroendocrine tumors (PanNETs) in the French PREDIR database, 1995–2022; median age at diagnosis 38 years
- Participants
- 121 patients259 PanNETs
- Outcome
- Proportion of patients with PanNET-related metastases
- Follow-up
- Median 89.5 months
Methodological detailsnational registry (French PREDIR database), PanNET surgical resection in 51 patients
- National registry (French PREDIR database)
- PanNET surgical resection in 51 patients
Main finding
As reported in the abstract
Patients with PanNET-related metastases
- Patients with metastases
- 29 patients (24%)
- Synchronous metastases
- 5
- Metachronous metastases
- 10
- Higher metastatic risk with larger PanNET size (best threshold 28 mm)
- P = .0089
- Higher metastatic risk with grade 2 PanNET
- P = .048
- Metastases had a pejorative prognostic impact
- P = .043
Observational data: associations, not effects.
Secondary outcomes
PV in VHL exon 1 vs other: PanNET size, metastatic disease and overall survival (OS)
- Larger PanNETs with PV in VHL exon 1
- P = 0.018
- More often metastatic disease with PV in VHL exon 1 (48% vs 11.5%)
- P < 0.001
- Trend toward shorter OS with PV in VHL exon 1
- P = 0.16
Original publication
Pancreatic Neuroendocrine Tumors in French VHL Mutation Carriers. J Clin Endocrinol Metab. Published 6 May 2024. doi:10.
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