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The Role of Cytotoxic Chemotherapy in Well-Differentiated Gastroenteropancreatic and Lung Neuroendocrine Tumors

  • Neuroendocrine Cancers (JR Strosberg, Section Editor)
  • Published:
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Opinion Statement

The treatment landscape of well-differentiated neuroendocrine tumors (NETs) has considerably expanded in recent years, and both somatostatin analogs, radiolabeled somatostatin analogs, everolimus, and sunitinib have been incorporated within the therapeutic armamentarium against these malignancies. Even in the context of multiple treatment options available, cytotoxic chemotherapy plays a pivotal role in the management of pancreatic NETs (panNETs), while its activity in midgut carcinoids and lung NETs is still debated. High response rates, ranging from 30 to 70%, have been consistently reported in studies of panNETs investigating streptozotocin-, temozolomide-, or platinum-based regimens, and an unprecedented prolongation of progression-free survival has been recently demonstrated in a prospective, randomized trial of capecitabine and temozolomide in patients with progressive panNETs. As a general principle, cytotoxic chemotherapy appears particularly appropriate in patients with bulky, symptomatic, or rapidly progressing tumors, especially of pancreatic origin, or in the salvage setting of NET patients who have failed alternative therapeutic options. Emerging evidence has also shown the potential efficacy of induction chemotherapy in patients with locally advanced or oligometastatic panNET, but prospective validation is needed before implementation of this approach in routine clinical practice. At present, there is no consensus on adjuvant therapy in pulmonary NETs, and differences between guideline recommendations at this regard mainly stem from the lack of high-level evidence. In the future, the identification of molecular biomarkers of response to chemotherapy might allow better patient preselection, thus leading to improved outcomes.

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Funding

This work was supported by Associazione Italiana per la Ricerca sul Cancro (AIRC; grant number no. 17536), Regione Puglia (Oncogenomic Project and Jonico-Salentino Project), and European NeuroEndocrine Tumor Society (ENETS; Excellence Academy Fellowship Grant 2016).

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Authors and Affiliations

  1. Department of Biomedical Sciences and Human Oncology, University of Bari “Aldo Moro”, Piazza Giulio Cesare, 11, 70124, Bari, Italy

    Mauro Cives MD, Eleonora Pelle’ MD, Davide Quaresmini MD, Barbara Mandriani PhD, Marco Tucci MD, PhD & Franco Silvestris MD

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  1. Mauro Cives MD
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  2. Eleonora Pelle’ MD
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  3. Davide Quaresmini MD
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  4. Barbara Mandriani PhD
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  5. Marco Tucci MD, PhD
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  6. Franco Silvestris MD
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Corresponding author

Correspondence to Franco Silvestris MD.

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Conflict of Interest

Mauro Cives has received compensation from Ipsen for service as a consultant. Eleonora Pelle’ declares that she has no conflict of interest. Davide Quaresmini declares that he has no conflict of interest. Barbara Mandriani declares that she has no conflict of interest. Marco Tucci declares that he has no conflict of interest. Franco Silvestris declares that he has no conflict of interest.

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This article is part of the Topical Collection on Neuroendocrine Cancers

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Cives, M., Pelle’, E., Quaresmini, D. et al. The Role of Cytotoxic Chemotherapy in Well-Differentiated Gastroenteropancreatic and Lung Neuroendocrine Tumors. Curr. Treat. Options in Oncol. 20, 72 (2019). https://doi.org/10.1007/s11864-019-0669-7

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