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Review
. 2021 Aug 27:11:653162.
doi: 10.3389/fonc.2021.653162. eCollection 2021.

Management of Large Cell Neuroendocrine Carcinoma

Virginia Corbett  1 , Susanne Arnold  2 , Lowell Anthony  2 , Aman Chauhan  2
Affiliations
Review

Management of Large Cell Neuroendocrine Carcinoma

Virginia Corbett et al. Front Oncol. .

Abstract

Background: Large cell neuroendocrine carcinoma (LCNEC) is a rare, aggressive cancer with a dismal prognosis. The majority of cases occur in the lung and the gastrointestinal tract; however, it can occur throughout the body. Recently advances in the understanding of the molecular underpinnings of this disease have paved the way for additional novel promising therapies. This review will discuss the current best evidence for management of LCNEC and new directions in the classification and treatment of this rare disease.

Methods: We performed a PubMed search for "Large cell neuroendocrine carcinoma" and "High grade neuroendocrine carcinoma." All titles were screened for relevance to the management of LCNEC. Papers were included based on relevance to the management of LCNEC.

Results: Papers were included reviewing both pulmonary and extra pulmonary LCNEC. We summarized the data driven best practices for the management of both early and advanced stage LCNEC. We describe emerging therapies with promising potential.

Discussion: LCNEC are rare and aggressive neoplasms. In advanced disease, the historical regimen of platinum based therapy in combination with etoposide or irinotecan remains among the commonly used first line therapies, however for extra thoracic LCNEC regimens like FOLFOX, FOLFOIRI and CAPTEM can also be used. Further effective and safe treatment options are desperately needed. Recently, new advances including a new understanding of the genetic subcategories of LCNEC and immunotherapy agents may guide further treatments.

Keywords: LCNEC; clinical management; future directions; high grade neuroendocrine carcinoma; large cell neuroendocrine carcinoma.

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Conflict of interest statement

AC has received research support in form of either grant or drug supply from BMS, Clovis, Lexicon, Nanopharmaceuticals, EMD Serono, ECS Progastrin. AC is also an advisor to Ipsen, Lexicon, Novartis, TerSera but has not accepted direct personal payments for advisory role. VC owns equity in Pfizer, BristolMyers Squibb, Seagen, and Viatris. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Spectrum of neuroendocrine neoplasms (NEN). This graphic demonstrates the spectrum of neuroendocrine tumors from low grade to high grade.
Figure 2
Figure 2
Distribution of LCNEC based on site of origin. This graphic shows the distribution of large cell neuroendocrine primary tumor locations.
See this image and copyright information in PMC

References

    1. Strosberg JR, Coppola D, Klimstra DS, Phan AT, Kulke MH, Wiseman GA, et al. The NANETS Consensus Guidelines for the Diagnosis and Management of Poorly Differentiated (High-Grade) Extrapulmonary Neuroendocrine Carcinomas. Pancreas (2010) 39(6):799–800. 10.1097/MPA.0b013e3181ebb56f - DOI - PMC - PubMed
    1. Burkeen G, Chauhan A, Agrawal R, Raiker R, Kolesar J, Anthony L, et al. Gynecologic Large Cell Neuroendocrine Carcinoma: A Review. Rare Tumors (2020) 12:2036361320968401. 10.1177/2036361320968401 - DOI - PMC - PubMed
    1. Tu X, Chang T, Nie L, Qiu S, Xu H, Huang Y, et al. Large Cell Neuroendocrine Carcinoma of the Prostate: A Systematic Review and Pooled Analysis. Urol Int (2019) 103(4):383–90. 10.1159/000499883 - DOI - PubMed
    1. Thompson ED, Stelow EB, Mills SE, Westra WH, Bishop JA. Large Cell Neuroendocrine Carcinoma of the Head and Neck: A Clinicopathologic Series of 10 Cases With an Emphasis on HPV Status. Am J Surg Pathol (2016) 40(4):471–8. 10.1097/PAS.0000000000000580 - DOI - PMC - PubMed
    1. Ogata S, Maeda R, Tomita M, Sato Y, Ayabe T, Nakamura K. Resected Thymic Large Cell Neuroendocrine Carcinoma: A Case Report and Review of the Literature. Int J Surg Case Rep (2019) 60:53–7. 10.1016/j.ijscr.2019.05.057 - DOI - PMC - PubMed

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