Rectal neuroendocrine carcinoma: case report of a rare entity and perspective review of promising agents

Article Details

Authors
Gabriela Antelo MD, Cinta Hierro MD, MSc, Juan Pablo Fernández MD, Eduardo Baena MD, Cristina Bugés MD, Laura Layos MD, José Luis Manzano MD, Mónica Caro MD, Ricard Mesia MD, PhD

Article Type
Review

DOI
10.7573/dic.2020-2-4

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Abstract

Neuroendocrine neoplasms (NENs) comprise a heterogeneous group of tumours, which can be classified into neuroendocrine tumours (NETs), neuroendocrine carcinomas (NECs) and mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs). To date, there is no consensus regarding the optimal therapy, which usually depends on the primary location and classification, according to morphological features of differentiation and proliferation rates. Nevertheless, multidisciplinary strategies combining medical treatments and locoregional strategies have yielded better efficacy results. Here, we report the case of a patient diagnosed with a nonfunctional rectal NECs with metastatic widespread to pelvic lymph nodes and bilateral lung metastases. The patient received three cycles of platinumetoposide, concomitantly with palliative radiotherapy. Although CT scan after three cycles showed a significant partial response, there was an early fatal progression only 3 months after having stopped systemic therapy. As formerly described in the literature, this case highlights the aggressive behaviour of NECs, rare tumours that often present in advanced stages at diagnosis. Lately, new insights into the molecular biology of NECs have unveiled the possibility of using novel drugs, such as targeted agents or immunotherapy, in molecularly selected subgroups of patients. In this review, we discuss the current management of this rare entity and provide an overview of the most relevant molecular findings, whilst illustrating the potential value that prescreening panels can offer, searching for actionable targets (MSI/dMMR, PD-L1, BRAFv600E) to guide therapy with promising agents that could fill a void in this disease.

Keywords: chemotherapy, epigenetic, gastro-enteropancreatic neuroendocrine neoplasms, immunotherapy, molecular alterations, neuroendocrine carcinomas, radiotherapy, targeted agents.

Citation: Antelo G, Hierro C, Fernández JP, Baena E, Bugés C, Layos L, Manzano JL, Caro M, Mesia R. Rectal neuroendocrine carcinoma: case report of a rare entity and perspective review of promising agents. Drugs in Context 2020; 9: 2020-2-4. DOI: 10.7573/dic.2020-2-4

Contributions: Both Dr Antelo and Dr Hierro are first coauthors and contributed equally. All authors contributed in the conception, design, data acquisition, writing and review of the manuscript. All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship for this article, take responsibility for the integrity of the work as a whole, and have given their approval for this version to be published.

Disclosure and potential conflicts of interest: Dr Antelo reports personal fees from Ipsen and Kyowa Kirin Farmacéutica S.L.U. (travel and accommodation). Dr Hierro reports grants from Bayer, personal fees from Ignyta and Lilly (speaker honoraria), and personal fees from Roche, Amgen and Merck (travel and accommodation). Dr Baena reports personal fees from JANSSEN-CILAG, S., and nonfinancial support from Kyowa Kirin Farmacéutica, S.L.U., Grünenthal España and Angelini Farmacéutica S.A. Dr Bugés reports personal fees from Sanofi, Amgen, Merck and Novartis (travel and accommodation). Dr Layos reports personal fees from Celgene and Sanofi (advisory role), and personal fees from Celgene, Sanofi, Merck, Amgen and Ipsen (travel and accommodation). Dr Mesia reports personal fees from Merck, MSD and BMS (speaker honoraria), and personal fees from Merck, MSD, BMS, Roche, Amgen, Nanobiotics, AztraZeneca, Pfizer and Bayer (advisory role). The other authors have nothing to disclose. The International Committee of Medical Journal Editors (ICMJE) Potential Conflicts of Interests form for the authors is available for download at: https://www.drugsincontext.com/wp-content/uploads/2020/04/dic.2020-2-4-COI.pdf

Acknowledgements: None.

Funding declaration: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. There was no funding associated with the preparation of this article.

Copyright: Copyright © 2020 Antelo G, Hierro C, Fernández JP, Baena E, Bugés C, Layos L, Manzano JL, Caro M, Mesia R. Published by Drugs in Context under Creative Commons License Deed CC BY NC ND 4.0 which allows anyone to copy, distribute and transmit the article provided it is properly attributed in the manner specified below. No commercial use without permission.

Correct attribution: Copyright © 2020 Antelo G, Hierro C, Fernández JP, Baena E, Bugés C, Layos L, Manzano JL, Caro M, Mesia R. https://doi.org/10.7573/dic.2020-2-4. Published by Drugs in Context under Creative Commons License Deed CC BY NC ND 4.0.

Article URL: https://www.drugsincontext.com/rectal-neuroendocrine-carcinoma:-case-report-of-a-rare-entity-and-perspective-review-of-promising-agents

Correspondence: Ricard Mesia, Medical Oncology Department, Catalan Institute of Oncology (ICO)-Badalona; Badalona-Applied Research Group in Oncology (B-ARGO)-Germans Trias i Pujol Institute (IGTP); Germans Trias i Pujol University Hospital (HUGTiP); Crta. de Canyet s/n, 08916 Badalona, Barcelona, Spain. rmesia@iconcologia.net

Provenance: invited; externally peer reviewed.

Submitted: 26 February 2020; Peer review comments to author: 8 April 2020; Revised manuscript received: 23 April 2020; Accepted: 24 April 2020; Publication date: 15 May 2020.

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