Reliability and Agreement of Radiological and Pathological Tumor Size in Patients with Multiple Endocrine Neoplasia Type 1-Related Pancreatic Neuroendocrine Tumors: Results from a Population-Based Cohort

Dirk-Jan van Beek, Helena M. Verkooijen, Sjoerd Nell, Bert A. Bonsing, Casper H. van Eijck, Harry van Goor, Frederik J. H. Hoogwater, Elisabeth J. M. Nieveen van Dijkum, Geert Kazemier, Cornelis H. C. Dejong, Lodewijk A. A. Brosens, Frank J. Wessels, Inne H. M. Borel Rinkes, Gerlof D. Valk, Menno R. Vriens*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Background: Pancreatic neuroendocrine tumors (pNETs) have a high prevalence in patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading cause of death. Tumor size is still regarded as the main prognostic factor and therefore used for surgical decision-making. We assessed reliability and agreement of radiological and pathological tumor size in a population-based cohort of patients with MEN1-related pNETs. Methods: Patients were selected from the Dutch MEN1 database if they had undergone a resection for a pNET between 2003 and 2018. Radiological (MRI, CT, and endoscopic ultrasonography [EUS]) and pathological tumor size were collected from patient records. Measures of agreement (Bland-Altman plots with limits of agreement [LoA] and absolute agreement) and reliability (intraclass correlation coefficients [ICC] and unweighted kappa) were calculated for continuous and categorized (< or >= 2 cm) pNET size. Results: In 73 included patients, the median radiological and pathological tumor sizes measured were 22 (3-160) and 21 (4-200) mm, respectively. Mean bias between radiological and pathological tumor size was -0.2 mm and LoA ranged from -12.9 to 12.6 mm. For the subgroups of MRI, CT, and EUS, LoA of radiological and pathological tumor size ranged from -9.6 to 10.9, -15.9 to 15.8, and -13.9 to 11.0, respectively. ICCs for the overall cohort, MRI, CT, and EUS were 0.80, 0.86, 0.75, and 0.76, respectively. Based on the 2 cm criterion, agreement was 81.5%; hence, 12 patients (18.5%) were classified differently between imaging and pathology. Absolute agreement and kappa values of MRI, CT, and EUS were 88.6, 85.7, and 75.0%, and 0.77, 0.71, and 0.50, respectively. Conclusion: Within a population-based cohort, MEN1-related pNET size was not systematically over- or underestimated on preoperative imaging. Based on agreement and reliability measures, MRI is the preferred imaging modality.

Original languageEnglish
Pages (from-to)705-717
Number of pages13
Issue number8
Publication statusPublished - Jul 2021


  • Magnetic resonance imaging
  • Endoscopic ultrasonography
  • Multiple endocrine neoplasia type 1
  • Pancreatic neuroendocrine tumor
  • Reliability
  • Agreement
  • Computed tomography
  • MEN1
  • EUS

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