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Enucleation and limited pancreatic resection provide long-term cure for insulinoma in multiple endocrine neoplasia type 1.
Neuroendocrinology. 2013; 98(4):290-8.N

Abstract

AIM

To assess the characteristics and long-term outcome after surgery in patients with multiple endocrine neoplasia type 1 (MEN1)-associated insulinoma.

METHODS

Retrospective analysis of prospectively collected data of MEN1 patients with organic hyperinsulinism at a tertiary referral center.

RESULTS

Thirteen (17%) of 74 patients with MEN1 had organic hyperinsulinism. The median age at diagnosis was 27 (range 9-48) years. In 7 patients insulinoma was the first manifestation of the syndrome. All patients had at least one pancreatic neuroendocrine neoplasm (pNEN) upon imaging, including CT, MRI or endoscopic ultrasonography. Seven patients had solitary lesions upon imaging, 4 patients had one dominant tumor with coexisting multiple small pNENs, and 2 patients had multiple lesions without dominance. Eight patients had limited resections (1 segmental resection, 7 enucleations), 4 subtotal distal pancreatectomies, and 1 patient a partial duodenopancreatectomy. There was no postoperative mortality. Six patients experienced complications, including pancreatic fistula in 5 patients. Pathological examination revealed median three (range 1-14) macro-pNENs sized between 6 and 40 mm, and a total of 14 potentially benign insulinomas were detected in the 13 patients. After median follow-up of 156 months, only 1 patient developed recurrent hyperinsulinism after initial enucleation. Twelve patients developed new pNENs in the pancreatic remnant and 4 patients underwent reoperations (3 for metastatic ZES, 1 for recurrent hyperinsulinism). One of 5 patients with an initial extended pancreatic resection developed insulin-dependent diabetes mellitus.

CONCLUSION

Enucleation and limited resection provide long-term cure for MEN1 insulinoma in patients with solitary or dominant tumors. Subtotal distal pancreatectomy should thus be preserved for patients with multiple pNENs without dominance given the risk of exocrine and endocrine pancreas insufficiency in the mostly young patients.

Authors+Show Affiliations

Department of Visceral, Thoracic and Vascular Surgery, Philipps University Marburg, Marburg, Germany.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

24356648

Citation

Bartsch, Detlef K., et al. "Enucleation and Limited Pancreatic Resection Provide Long-term Cure for Insulinoma in Multiple Endocrine Neoplasia Type 1." Neuroendocrinology, vol. 98, no. 4, 2013, pp. 290-8.
Bartsch DK, Albers M, Knoop R, et al. Enucleation and limited pancreatic resection provide long-term cure for insulinoma in multiple endocrine neoplasia type 1. Neuroendocrinology. 2013;98(4):290-8.
Bartsch, D. K., Albers, M., Knoop, R., Kann, P. H., Fendrich, V., & Waldmann, J. (2013). Enucleation and limited pancreatic resection provide long-term cure for insulinoma in multiple endocrine neoplasia type 1. Neuroendocrinology, 98(4), 290-8. https://doi.org/10.1159/000357779
Bartsch DK, et al. Enucleation and Limited Pancreatic Resection Provide Long-term Cure for Insulinoma in Multiple Endocrine Neoplasia Type 1. Neuroendocrinology. 2013;98(4):290-8. PubMed PMID: 24356648.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Enucleation and limited pancreatic resection provide long-term cure for insulinoma in multiple endocrine neoplasia type 1. AU - Bartsch,Detlef K, AU - Albers,Max, AU - Knoop,Richard, AU - Kann,Peter H, AU - Fendrich,Volker, AU - Waldmann,Jens, Y1 - 2013/12/17/ PY - 2013/08/23/received PY - 2013/12/06/accepted PY - 2013/12/21/entrez PY - 2013/12/21/pubmed PY - 2014/12/15/medline SP - 290 EP - 8 JF - Neuroendocrinology JO - Neuroendocrinology VL - 98 IS - 4 N2 - AIM: To assess the characteristics and long-term outcome after surgery in patients with multiple endocrine neoplasia type 1 (MEN1)-associated insulinoma. METHODS: Retrospective analysis of prospectively collected data of MEN1 patients with organic hyperinsulinism at a tertiary referral center. RESULTS: Thirteen (17%) of 74 patients with MEN1 had organic hyperinsulinism. The median age at diagnosis was 27 (range 9-48) years. In 7 patients insulinoma was the first manifestation of the syndrome. All patients had at least one pancreatic neuroendocrine neoplasm (pNEN) upon imaging, including CT, MRI or endoscopic ultrasonography. Seven patients had solitary lesions upon imaging, 4 patients had one dominant tumor with coexisting multiple small pNENs, and 2 patients had multiple lesions without dominance. Eight patients had limited resections (1 segmental resection, 7 enucleations), 4 subtotal distal pancreatectomies, and 1 patient a partial duodenopancreatectomy. There was no postoperative mortality. Six patients experienced complications, including pancreatic fistula in 5 patients. Pathological examination revealed median three (range 1-14) macro-pNENs sized between 6 and 40 mm, and a total of 14 potentially benign insulinomas were detected in the 13 patients. After median follow-up of 156 months, only 1 patient developed recurrent hyperinsulinism after initial enucleation. Twelve patients developed new pNENs in the pancreatic remnant and 4 patients underwent reoperations (3 for metastatic ZES, 1 for recurrent hyperinsulinism). One of 5 patients with an initial extended pancreatic resection developed insulin-dependent diabetes mellitus. CONCLUSION: Enucleation and limited resection provide long-term cure for MEN1 insulinoma in patients with solitary or dominant tumors. Subtotal distal pancreatectomy should thus be preserved for patients with multiple pNENs without dominance given the risk of exocrine and endocrine pancreas insufficiency in the mostly young patients. SN - 1423-0194 UR - https://www.unboundmedicine.com/medline/citation/24356648/Enucleation_and_limited_pancreatic_resection_provide_long_term_cure_for_insulinoma_in_multiple_endocrine_neoplasia_type_1_ L2 - https://www.karger.com?DOI=10.1159/000357779 DB - PRIME DP - Unbound Medicine ER -