Long-term results of the surgical management of insulinoma patients with MEN1: a Groupe d'étude des Tumeurs Endocrines (GTE) retrospective study

Abstract

Objective: Management of insulinomas in the context of MEN1 remains poorly studied. The aim of this study was to evaluate long-term results of various surgical approaches in a large cohort of insulinoma-MEN1 patients. Design and methods: Consecutive insulinoma-MEN1 patients operated on for a nonmetastatic insulinoma between 1957 and 2010 were retrospectively selected from the MEN1 database of the French Endocrine Tumor Group. The type of surgery was categorized as distal pancreatectomy (DP), total pancreatectomy/cephalic duodenopancreatectomy (TP/CDP), or enucleation (E). Primary endpoint was time until recurrence of hypoglycemia after initial surgery. Secondary endpoints were post-operative complications. Results: The study included 73 patients (median ageZ28 years). Surgical procedures were DP (nZ46), TP/CDP (nZ9), or E (nZ18). After a median post-operative follow-up of 9.0 years (inter-quartile range (IQR): 2.5-16.5 years), 60/73 patients (82.2%) remained hypoglycemia free. E and TP/CDP were associated with a higher risk of recurrent hypoglycemia episodes (unadjusted hazard ratio: 6.18 ((95% CI: 1.54-24.8); PZ0.010) for E vs DP and 9.51 ((95% CI: 1.85-48.8); PZ0.007) for TP/CDP vs DP. After adjustment for International Union against Cancer pTNM classification, enucleation remained significantly associated with a higher probability of recurrence. Long-term complications had occurred in 20 (43.5%) patients European Journal of EndocrinologyClinical Study D Vezzosi and others Surgery in insulinoma-MEN1 patients

The future: surgical advances in MEN1 therapeutic approaches and management strategies

“…The overall cure rate was 82.2% and reached 91.3% with DPR after median follow-up of 9 years. Thus, DPR is associated with a low risk of hypoglycemic symptom recurrence, but because of the low morbidity, EN alone might be considered an alternative in patients with MEN1 (Vezzosi et al 2015). In EN, an open or laparoscopic approach did not significantly alter the risk of recurrence of hypoglycemia symptoms.…”

“…2) (Vezzosi et al 2015). No recurrences were reported after DPR combined with EN for additional tumors in the pancreatic head.…”

“…CDP, cephalic duodenopancreatectomy; TP, total pancreatectomy; DP, distal pancreatectomy; E, enucleation alone. Adapted, with permission, from Vezzosi et al (2015). Copyright 2015 European Society of Endocrinology.…”

Multiple endocrine neoplasia syndrome type 1: institution, management, and data analysis of a nationwide multicenter patient database

“…The collection of clinical, biochemical, and genetic characteristics of unselected patients with rare disorders, such as MEN1, in multicentre-nationwide registries or databases, is a useful approach to increase knowledge of epidemiological aspects of the disease and the natural course and prognosis of single manifestations of the syndrome [16][17][18][19][20].…”

Recent Advances in the Management of Endocrine Malignancies Associated with Hereditary Hyperparathyroidism Syndromes

“…Parenchymasparing surgery, such as enucleation, risks disease understaging and pancreatic duct leak or fistula formation (50%). More aggressive surgery, such as distal pancreatectomy, which is indicated in patients with large and multiple tumors, confers a high risk of endocrine and exocrine pancreatic insufficiency [81][82][83]. Enucleation can be combined with distal pancreatectomy as needed.…”