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Review
. 2023 Jul;24(7):725-741.
doi: 10.1007/s11864-023-01085-0. Epub 2023 Apr 27.

Management of Functional Pancreatic Neuroendocrine Neoplasms

Ludovica Magi  1 , Matteo Marasco  1 , Maria Rinzivillo  1 , Antongiulio Faggiano  2   3 , Francesco Panzuto  4   5
Affiliations
Review

Management of Functional Pancreatic Neuroendocrine Neoplasms

Ludovica Magi et al. Curr Treat Options Oncol. 2023 Jul.

Abstract

Functional pancreatic neuroendocrine neoplasms (pNENs) are rare and heterogeneous diseases in terms of both clinical and pathological aspects. These tumors secrete hormones or peptides, which may cause a wide variety of symptoms related to a clinical syndrome. The management of functional pNENs is still challenging for clinicians due to the need to control both tumor growth and specific symptoms. Surgery remains the cornerstone in the management of local disease because it can definitively cure the patient. However, when the disease is not resectable, a broad spectrum of therapeutic options, including locoregional therapy, somatostatin analogs (SSAs), targeted therapies, peptide-receptor radionuclide therapy (PRRT), and chemotherapy, are available. The present review summarizes the main key issues regarding the clinical management of these tumors, providing a specific highlight on their therapeutic approach.

Keywords: Gastrinoma; Insulinoma; Neuroendocrine tumors; Somatostatin analogs; Treatment.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Fig. 1
Fig. 1
Management of functional pancreatic neuroendocrine neoplasms. This figure represents a practical algorithm for the management of functional pNENs. Abbreviations: RFA, radiofrequency ablation; MEN 1, multiple endocrine neoplasia syndrome type 1; TAE, transarterial embolization; TACE, transarterial chemoembolization; TARE, transarterial radioembolization; PRRT, peptide radio receptor therapy; SSAs, somatostatin analogs; PPIs, proton pump inhibitors; IFN-a, interferon alpha.
See this image and copyright information in PMC

References

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