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  • Year in Review
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NEUROENDOCRINE TUMOURS IN 2020

Diagnostic and therapeutic advances in neuroendocrine tumours

Neuroendocrine neoplasms are derived from the diffuse endocrine system and represent a spectrum of tumours with a diverse range of molecular abnormalities, functionality and anatomical locations. Here, some key advances in molecular diagnosis, functional imaging and therapeutic strategies that have been published in 2020 are discussed.

Key advances

  • The NETest could serve as a valid biomarker in diagnosis, follow up and monitoring response to treatment of neuroendocrine neoplasms (NENs)2,3.

  • The 68Ga somatostatin analogue PET-CT has a higher diagnostic accuracy for well-differentiated grade I and II NENs than 18F-fluorodeoxyglucose PET-CT4.

  • Peptide receptor radionuclide therapy with 177Lu DOTA-TATE has improved outcomes for patients with 68Ga-DOTA-somatostatin receptor-positive advanced well-differentiated NENs5.

  • Findings from immunotherapy trials for patients with NENs have been somewhat disappointing in patients with low-grade NENs. However, analysis of immune-related genes in pancreatic NENs suggests that patients might be stratified by molecular subtypes and immune-related gene expression9.

  • Surgery with a radical intent could be a valid option for patients with grade III gastroenteropancreatic-NETs with Ki67 <55%10.

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Fig. 1: 68Ga DOTA-TATE PET-CT scan of a patient with primary bronchial neuroendocrine neoplasm and multiple liver, lymph node and bone secondary tumour foci.

References

  1. Malczewska, A. et al. An assessment of circulating chromogranin A as a biomarker of bronchopulmonary neuroendocrine neoplasia: a systematic review and meta-analysis. Neuroendocrinology 110, 198–216 (2020).

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  2. Öberg, K. et al. A meta-analysis of the accuracy of a neuroendocrine tumor mRNA genomic biomarker (NETest) in blood. Ann. Oncol. 31, 202–212 (2020).

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  3. Bodei, L. et al. PRRT neuroendocrine tumor response monitored using circulating transcript analysis: the NETest. Eu. J. Nucl. Med. Mol. Imaging 47, 895–906 (2020).

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  4. Liu, X. et al. Comparison of Gallium-somatostatin receptor and 18F-fluorodeoxyglucose positron emission tomography in the diagnosis of neuroendocrine tumours: A systematic review and meta-analysis. Hell. J. Nucl. Med. 23, 188–200 (2020).

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  5. Saravana-Bawan, B., Bajwa, A., Paterson, J., McEwan, A. J. B. & McMullen, T. P. W. Efficacy of 177Lu peptide receptor radionuclide therapy for the treatment of neuroendocrine tumors a meta-analysis. Clin. Nucl. Med. 44, 719–727 (2019).

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  6. Mehnert, J. M. et al. Pembrolizumab for the treatment of programmed death–ligand 1–positive advanced carcinoid or pancreatic neuroendocrine tumors: Results from the KEYNOTE-028 study. Cancer 126, 3021–3030 (2020).

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  7. Patel, S. P. et al. A Phase II Basket Trial of Dual Anti–CTLA-4 and Anti–PD-1 Blockade in Rare Tumors (DART SWOG 1609) in Patients with Nonpancreatic Neuroendocrine Tumors. Clin. Cancer Res. 26, 2290–2296 (2020).

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  8. Capdevila, J. et al. 1157O A multi-cohort phase II study of durvalumab plus tremelimumab for the treatment of patients (pts) with advanced neuroendocrine neoplasms (NENs) of gastroenteropancreatic or lung origin: The DUNE trial (GETNE 1601). Ann. Oncol. 31, S770–S771 (2020).

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  9. Young, K. et al. Immune landscape, evolution, hypoxia-mediated viral mimicry pathways and therapeutic potential in molecular subtypes of pancreatic neuroendocrine tumours. Gut https://doi.org/10.1136/gutjnl-2020-321016 (2020).

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  10. Merola, E. et al. Surgery with Radical Intent: Is There an Indication for G3 Neuroendocrine Neoplasms? Ann. Surg. Oncol. 27, 1348–1355 (2020).

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Correspondence to Martyn E. Caplin.

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Competing interests

M.E.C. has received speaker honoraria and research funding from AAA-Novartis, Ipsen and Pfizer. G.M.R. declares no competing interests.

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Caplin, M.E., Ratnayake, G.M. Diagnostic and therapeutic advances in neuroendocrine tumours. Nat Rev Endocrinol 17, 81–82 (2021). https://doi.org/10.1038/s41574-020-00458-x

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