Abstract

Small cell carcinoma is a type of highly aggressive poorly differentiated neuroendocrine tumor that can arise from multiple organs, including but not limited to bronchial tissue, pancreas, gastrointestinal tract, and genitourinary system. The most commonly studied type is small cell lung cancer (SCLC) which carries the worst prognosis among lung cancers. After multiple promising clinical trials, the National Comprehensive Cancer Network has recently added atezolizumab and durvalumab in combination with platinum-based chemotherapy/etoposide to the first-line treatment regimen for extensive-stage SCLC (ES-SCLC). Meanwhile, the recommended treatment for extrapulmonary small cell carcinoma (EPSCC) remains unchanged. In this review, we try to explore the role of immunotherapy in the treatment of EPSCC.

Highlights

  • BackgroundSmall cell carcinomas are poorly differentiated highly aggressive neuroendocrine tumors that can arise from a variety of epithelial tissues, with the most common being the bronchial tree, constituting up to 15% of lung cancers [1]

  • Extrapulmonary small cell carcinoma (EPSCC) are very rare but highly aggressive tumors, with most cases presenting with widespread metastatic disease

  • The active Phase II trial (NCT03866382) that started on April, 12,2019 is testing the effectiveness of two immunotherapy drugs with cabozantinib in patients with rare metastatic genitourinary tumors including small cell carcinoma of the bladder, this trial is expected to be completed in February 2023

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Summary

Introduction

Small cell carcinomas are poorly differentiated highly aggressive neuroendocrine tumors that can arise from a variety of epithelial tissues, with the most common being the bronchial tree, constituting up to 15% of lung cancers [1]. Despite the fact that her tumor showed absent PD-L1 expression, Radiographic imaging demonstrated a decrease in the size of all target lesions after two doses of treatment [27] Another case reported a good response to nivolumab in a patient with metastatic small cell carcinoma. The active Phase II trial (NCT03866382) that started on April, 12,2019 is testing the effectiveness of two immunotherapy drugs (nivolumab and ipilimumab) with cabozantinib in patients with rare metastatic genitourinary tumors including small cell carcinoma of the bladder, this trial is expected to be completed in February 2023 Another clinical trial (NCT03910660) looking into the use of the new investigational drug BXCL701 which is an orally-available systemic innate immunity activator, in combination with pembrolizumab for treatment of metastatic small cell carcinoma of the prostate, this study is expected to conclude in April 2022. There are currently no other trials involving EPSCC of other tissue origins

Conclusions
Disclosures
Findings
Travis WD
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