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Review
. 2021 Jun 10;22(8):68.
doi: 10.1007/s11864-021-00866-9.

Systemic Treatment of Gastroenteropancreatic Neuroendocrine Carcinoma

Kazhan Mollazadegan  1 , Staffan Welin  2 , Joakim Crona  3
Affiliations
Review

Systemic Treatment of Gastroenteropancreatic Neuroendocrine Carcinoma

Kazhan Mollazadegan et al. Curr Treat Options Oncol. .

Abstract

Treatment recommendations for advanced gastroenteropancreatic neuroendocrine carcinomas (GEP-NEC) are based on uncontrolled, mainly retrospective data. Chemotherapy can offer palliative relief, but long-lasting complete responses or cures are rare. The European Neuroendocrine Tumour Society (ENETS) and European Society for Medical Oncology (ESMO) recommend platinum-based chemotherapy as first-line treatment. This has been the golden standard since the late 1980s and has been evaluated in mostly retrospective clinical studies. However, progression is inevitable for most patients. Unfortunately, data on effective second-line treatment options are scant, and ENETS and ESMO recommendations propose fluorouracil- or temozolomide-based chemotherapy schedules. As such, there is a huge unmet need for improved care. Improved knowledge on GEP-NEC biology may provide a pathway towards more effective interventions including chemotherapy, targeted gene therapy, peptide receptor radionuclide therapy, as well as immune checkpoint inhibitors. The review summarises this current state of the art as well as the most promising developments for systemic therapy in GEP-NEC patients.

Keywords: Extra pulmonary; Gastroenteropancreatic carcinoma; High-grade; Neuroendocrine carcinoma; Poorly differentiated; Systemic treatment.

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Conflict of interest statement

KM declares no conflicts of interest. JC received lecture honoraria from Novartis and IPSEN as well as educational honoraria from NET Connect (funded by IPSEN).

References

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