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J Immunother Cancer. 2017 Sep 19;5(1):75. doi: 10.1186/s40425-017-0281-y.

Checkpoint inhibitor is active against large cell neuroendocrine carcinoma with high tumor mutation burden.

Author information

1
Department of Medicine, University of California, 1600 Divisadero Street, San Francisco, California, 94115, USA. victoria.wang@ucsf.edu.
2
Department of Pathology, University of California, San Francisco, USA.
3
Foundation Medicine, Cambridge, Massachusetts, USA.
4
Department of Medicine, University of California, 1600 Divisadero Street, San Francisco, California, 94115, USA.
5
Department of Surgery, University of California, San Francisco, USA.

Abstract

BACKGROUND:

Large cell neuroendocrine tumor (LCNEC) of the lung is a rare and aggressive tumor similar to small cell lung cancer (SCLC). Thus, it is often treated similarly to SCLC in the front-line setting with a platinum doublet. However, treatment for patients beyond the first line remains undefined.

CASE PRESENTATION:

We report the case of a patient with stage IB LCNEC (PD-L1 negative but positive for PD-L1 amplification and tumor mutation burden high) who progressed after adjuvant chemotherapy after surgery and subsequent therapy with an antibody drug conjugate targeting a neuroendocrine-specific cell surface marker but achieved a significant and durable response with pembrolizumab, a humanized IgG4 monoclonal anti-PD-1 antibody.

CONCLUSIONS:

Immunotherapy with checkpoint inhibitors is an effective treatment option for patients with metastatic LCNEC, even if PD-L1 expression is negative.

KEYWORDS:

Immunotherapy; Large cell neuroendocrine tumor; PD-L1; Pembrolizumab; Tumor mutation burden

PMID:
28923100
PMCID:
PMC5604145
DOI:
10.1186/s40425-017-0281-y
[Indexed for MEDLINE]
Free PMC Article

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