Diagnostic and management strategies for pNETs in Von Hippel–Lindau: a systematic review

in Endocrine-Related Cancer
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  • 1 Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, the Netherlands
  • 2 Dutch VHL Organization, Utrecht, the Netherlands

Correspondence should be addressed to R S van Leeuwaarde: r.vanleeuwaarde@umcutrecht.nl
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Pancreatic neuroendocrine tumors (pNETs) in Von Hippel–Lindau (VHL) disease have a relatively good prognosis. However, a subset of pNETs metastasize and significantly contribute to VHL-related mortality. Evidence-based guidelines are needed for timely detection, management and intervention of these tumors. However, the value of several diagnostic tools is controversial, and evidence-based management strategies are lacking. This systematic review aims to update current literature on diagnostic and management strategies of pNETs in VHL and proposes evidence-based recommendations. The databases of PubMed/Medline, Embase and Web of Science were systematically searched to identify relevant studies. Studies were screened independently and cross-checked by two authors to assess eligibility for inclusion. Eighty-four articles were eligible for full text reading, and thirteen were critically appraised using the modified Quality Assessment of Diagnostic Accuracy Studies or modified Quality in Prognostic Studies tool. Six studies assessed the diagnostic value of imaging modalities, five focused on the optimal timing for surgical intervention, and one article studied the growth rate of pNETs. Quality of the available evidence was determined using the Grading of Recommendations, Assessment, Development and Evaluations tool. Studies recommended CT or MRI as the primary screening modalities for pNETs. For detection of metastases, 68Gallium-DOTATATE/TOC PET/CT is advised. For pNETs <2 cm a watch-and-wait approach is recommended, while for pNETs ≥2.5 cm surgical resection is advised. Due to limited data, no strong recommendations on surveillance could be proposed.

Supplementary Materials

    • Supplementary Figure 1A. Flowchart - Diagnostic value of Imaging modalities
    • Supplementary Figure 1B. Flowchart &#x2013; Tumor growth and age-related penetrance
    • Supplementary Figure 1C. Flowchart &#x2013; Optimal tumor size for surgery
    • Supplementary Table 2A. Characteristics and Risk of Bias of included studies assessing
    • Supplementary Table 2B. Characteristics and Risk of Bias of included studies evaluating the optimal size for surgical intervention in VHL related pNETs.

 

Society for Endocrinology

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