Management of MEN1 Related Nonfunctioning Pancreatic NETs: A Shifting Paradigm: Results From the DutchMEN1 Study Group
Nell, Sjoerd; Verkooijen, Helena M.; 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.
(2018) Annals of Surgery, volume 267, issue 6, pp. 1155 - 1160
(Article)
Abstract
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.
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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.
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Keywords: Adult, Female, Humans, Liver Neoplasms/prevention & control, Male, Multiple Endocrine Neoplasia Type 1/complications, Multiple endocrine neoplasia type 1, Pancreatic Neoplasms/complications, Pancreatic neuroendocrine tumors, Proportional Hazards Models, Watchful Waiting, oncology, surgery, survival, Surgery, Journal Article, Research Support, Non-U.S. Gov't
ISSN: 0003-4932
Publisher: Lippincott Williams and Wilkins
Note: Publisher Copyright: Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved. Copyright: Copyright 2018 Elsevier B.V., All rights reserved.
(Peer reviewed)