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Results of Duodenopancreatic Reoperations in Multiple Endocrine Neoplasia Type 1

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Abstract

Background

To evaluate the outcome of duodenopancreatic reoperations in patients with multiple endocrine neoplasia type 1 (MEN1).

Methods

MEN1 patients who underwent reoperations for duodenopancreatic neuroendocrine neoplasms (dpNENs) were retrieved from a prospective database and retrospectively analyzed.

Results

Twelve of 101 MEN1 patients underwent up to three reoperations, resulting in a total of 18 reoperations for dpNEN recurrence. Patients initially underwent either formal pancreatic resections (n = 7), enucleations (n = 3), or duodenotomy with lymphadenectomy for either NF-pNEN (seven patients), Zollinger–Ellison syndrome (ZES, three patients), organic hyperinsulinism (one patient) or VIPoma (one patient). Six patients had malignant dpNENs with lymph node (n = 5) and/or liver metastases (n = 2). The indication of reoperations was NF-pNEN (five patients), ZES (five patients), organic hyperinsulinism (one patient), and recurrent VIPoma (one patient). Median time to first reoperation was 67.5 (range 6–251) months. Five patients required a second duodenopancreatic reoperation for 60–384 months after initial surgery, and one patient underwent a third reoperation after 249 months. The rate of complications (Clavien–Dindo ≥3) was 28%. Four patients required completion pancreatectomy. Six patients developed pancreoprivic diabetes. After a median follow-up of 18 (6–34) years after initial surgery, ten of 12 patients are alive, one died of metastatic pancreatic VIPoma, and one died of metastatic thymic NEN.

Conclusion

Reoperations are frequently necessary for dpNEN in MEN1 patients, but are not associated with an increased perioperative morbidity in specialized centers. Organ-sparing resections should be preferred as initial duodenopancreatic procedures to maintain pancreatic function and avoid completion pancreatectomy.

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Acknowledgements

We thank all MEN1 patients participating in our controlled screening program.

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Authors and Affiliations

  1. Department of Visceral-, Thoracic- and Vascular Surgery, Philipps University of Marburg, Baldingerstr, 35043, Marburg, Germany

    Max B. Albers, Jerena Manoharan, Carmen Bollmann, Maximilian P. Chlosta, Katharina Holzer & Detlef K. Bartsch

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  1. Max B. Albers
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  2. Jerena Manoharan
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  3. Carmen Bollmann
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Correspondence to Max B. Albers.

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Albers, M.B., Manoharan, J., Bollmann, C. et al. Results of Duodenopancreatic Reoperations in Multiple Endocrine Neoplasia Type 1. World J Surg 43, 552–558 (2019). https://doi.org/10.1007/s00268-018-4809-1

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  • DOI: https://doi.org/10.1007/s00268-018-4809-1

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