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. 2019 Feb 22;14(1):54.
doi: 10.1186/s13023-019-1034-4.

Intertumor heterogeneity in 60 pancreatic neuroendocrine tumors associated with multiple endocrine neoplasia type 1

Andreas Selberherr  1 , Oskar Koperek  2 , Philipp Riss  3 , Christian Scheuba  3 , Martin B Niederle  4 , Reto Kaderli  3 , Aurel Perren  5 , Bruno Niederle  3
Affiliations

Intertumor heterogeneity in 60 pancreatic neuroendocrine tumors associated with multiple endocrine neoplasia type 1

Andreas Selberherr et al. Orphanet J Rare Dis. .

Abstract

Background: Patients with multiple endocrine neoplasia type 1 (MEN-1) develop multiple pancreatic neuroendocrine neoplasias (PNENs). Size at diagnosis and growth during follow-up are crucial parameters. According to the WHO 2017, grading is another important parameter. The impact of grading compared to size (WHO 2000) on the clinical course needs to be evaluated.

Methods: Sixty PNENs of six patients with MEN-1 were retrospectively evaluated.

Results: Fifty-one tumors with a diameter of < 20 mm were graded as G1. Two of 9 tumors with diameters of ≥20 mm were graded as G2. Tumor size of ≥20 mm correlated significantly with higher proliferation (p = 0.000617). Lymph node metastases were documented in two patients with a total of 19 tumors. In one patient, all 13 tumors (diameter: 0.4 to 100 mm) were classified as G1. However, metastases were documented in 9/29 lymph nodes. In the other patient, 5 tumors (3.5 to 20 mm) were classified as G1. The sixth tumor (30 mm) was classified as G2 (Ki-67: 8%). Metastases were revealed in 2/20 lymph nodes.

Conclusions: Tumor size of ≥20 mm seems to correlate with more aggressive MEN-1 related pancreatic disease, regardless of individual proliferation. Tumors ≥20 mm and tumors graded as G2 should be treated surgically regardless of their size.

Keywords: Intertumor heterogeneity; MEN-1; Multiple endocrine neoplasia; NET; Pancreatic neuroendocrine tumors.

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Conflict of interest statement

Ethics approval and consent to participate

All procedures performed in this study involving human material were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in the study.

Consent for publication

Consent for publication was obtained from all participants.

Competing interests

The authors declare that they have no competing interests.

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Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
CT-image of two large tumors (80 mm and 40 mm) of patient A that were both immunohistochemically positive for VIP
See this image and copyright information in PMC

References

    1. Doherty GM, Thompson NW. Multiple endocrine neoplasia type 1: duodenopancreatic tumours. J Intern Med. 2003;253(6):590–598. doi: 10.1046/j.1365-2796.2003.01163.x. - DOI - PubMed
    1. Gauger PG, Thompson NW. Early surgical intervention and strategy in patients with multiple endocrine neoplasia type 1. Best Pract. Res. Clin. Endocrinol. Metab. 2001;15(2):213–223. doi: 10.1053/beem.2001.0136. - DOI - PubMed
    1. Kouvaraki MA, Shapiro SE, Cote GJ, et al. Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. World J Surg. 2006;30(5):643–653. doi: 10.1007/s00268-006-0360-y. - DOI - PubMed
    1. Akerstrom G, Hessman O, Skogseid B. Timing and extent of surgery in symptomatic and asymptomatic neuroendocrine tumors of the pancreas in MEN 1. Langenbecks Arch Surg. 2002;386(8):558–569. doi: 10.1007/s00423-001-0274-6. - DOI - PubMed
    1. Gauger PG, Doherty GM, Broome JT, et al. Completion pancreatectomy and duodenectomy for recurrent MEN-1 pancreaticoduodenal endocrine neoplasms. Surgery. 2009;146(4):801–806. doi: 10.1016/j.surg.2009.06.038. - DOI - PubMed