Management of MEN1 Related Nonfunctioning Pancreatic NETs: A Shifting Paradigm:Results From the DutchMEN1 Study Group

OBJECTIVE: To assess if surgery for Multiple Endocrine Neoplasia type 1 (MEN1) related nonfunctioning pancreatic neuroendocrine tumors (NF-pNETs) is effective for improving overall survival and preventing liver metastasis. BACKGROUND: MEN1 leads to multiple early-onset NF-pNETs. The evidence base for guiding the difficult decision who and when to operate is meager. METHODS: MEN1 patients diagnosed with NF-pNETs between 1990 and 2014 were selected from the DutchMEN1 Study Group database, including > 90% of the Dutch MEN1 population. The effect of surgery was estimated using time-dependent Co... Mehr ...

Verfasser: Nell, Sjoerd
Pieterman, Carolina R C
de Herder, Wouter W
Hermus, Ad R
Dekkers, Olaf M
van der Horst-Schrivers, Anouk N
Drent, Madeleine L
Bisschop, Peter H
Havekes, Bas
Borel Rinkes, Inne H M
Vriens, Menno R
Valk, Gerlof D
Dokumenttyp: Artikel
Erscheinungsdatum: 2018
Reihe/Periodikum: Nell , S , Pieterman , C R C , de Herder , W W , Hermus , A R , Dekkers , O M , van der Horst-Schrivers , A N , Drent , M L , Bisschop , P H , Havekes , B , Borel Rinkes , I H M , Vriens , M R & Valk , G D 2018 , ' Management of MEN1 Related Nonfunctioning Pancreatic NETs: A Shifting Paradigm : Results From the DutchMEN1 Study Group ' , Annals of Surgery , vol. 267 , no. 6 , pp. 1155-1160 . https://doi.org/10.1097/SLA.0000000000002183
Schlagwörter: Journal Article / /dk/atira/pure/sustainabledevelopmentgoals/good_health_and_well_being / name=SDG 3 - Good Health and Well-being
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-26687222
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : https://research.vu.nl/en/publications/ca912b8d-46db-444c-95a5-1e48e43f9538

OBJECTIVE: To assess if surgery for Multiple Endocrine Neoplasia type 1 (MEN1) related nonfunctioning pancreatic neuroendocrine tumors (NF-pNETs) is effective for improving overall survival and preventing liver metastasis. BACKGROUND: MEN1 leads to multiple early-onset NF-pNETs. The evidence base for guiding the difficult decision who and when to operate is meager. METHODS: MEN1 patients diagnosed with NF-pNETs between 1990 and 2014 were selected from the DutchMEN1 Study Group database, including > 90% of the Dutch MEN1 population. The effect of surgery was estimated using time-dependent Cox analysis with propensity score restriction and adjustment. RESULTS: Of the 152 patients, 53 underwent surgery and 99 were managed by watchful waiting. In the surgery group, tumors were larger and faster-growing, patients were younger, more often male, and were more often treated in centers that operated more frequently. Surgery for NF-pNETs was not associated with a significantly lower risk of liver metastases or death, [adjusted hazard ratio (HR) = 0.73 (0.25-2.11)]. Adjusted HR's after stratification by tumor size were: NF-pNETs <2 cm = 2.04 (0.31-13.59) and NF-pNETs 2-3 cm = 1.38 (0.09-20.31). Five out of the 6 patients with NF-pNETs >3 cm managed by watchful waiting developed liver metastases or died compared with 6 out of the 16 patients who underwent surgery. CONCLUSIONS: MEN1 patients with NF-pNETs <2 cm can be managed by watchful waiting, hereby avoiding major surgery without loss of oncological safety. The beneficial effect of a surgery in NF-pNETs 2 to 3 cm requires further research. In patients with NF-pNETs >3 cm, watchful waiting seems not advisable.