Distal pancreatectomy for neuroendocrine neoplasms in patients with multiple endocrine neoplasia type 1.

BACKGROUND

To analyze the outcomes of multiple endocrine neoplasia type 1 (MEN1) patients after distal pancreatectomy (DP) for pancreatic neuroendocrine neoplasm (P-NENs) with a specific attention to postoperative complications and long-term survival.

METHODS

MEN1 patients from the French MEN1 cohort who underwent DP for P-NENs between 1990 and 2022 were included in the study. Early and late postoperative complications, secretory control, pancreatic disease-free and overall survival were assessed.

RESULTS

Out of 62 patients included, 41 (66%) underwent DP for a non-functioning P-NENs, 14 (23%) for insulinomas, 4 (6%) for a VIPoma and 3 (5%) for a glucagonoma. Median follow-up was 84 months (range: 12-335). No death within the 90 postoperative days was observed. Clavien-Dindo grade III-V postoperative complications occurred in 16% of the patients (n=11). Fourteen patients (23%) developed pancreatic fistulas. Twenty-four patients (39%) developed diabetes mellitus and 8 patients (13%) developed pancreatic exocrine insufficiency. The secretory control rate was 90%. Mean age at death was 56±10.9years. Five-year and ten-year overall survival was 93.0% [CI (85-100)], and 86.7% [CI (76.2-98.5)]. Ten-year liver metastasis-free survival was significantly shorter when DP was performed for non-functioning P-NENs compared to insulinomas 60.4% [CI (47-79)] versus 83.3% [CI (68-87)] (P=0.007).

CONCLUSION

Distal pancreatectomy in MEN1 patients is a safe surgical procedure with an acceptable morbidity and a very low postoperative mortality. Further studies are needed to standardize the extent of lymph node removal when distal pancreatectomy is performed in MEN-1 patients.