Case report: Robust response to sintilimab in advanced distal cholangiocarcinoma with PD-L1 expression and DNA damage repair

Cholangiocarcinoma (CCA) is a highly heterogeneous tumor that occurs in the bile duct epithelium; adenosquamous carcinoma is a rare pathological subtype of CCA. The clinical treatment of patients with metastatic distal CCA poses significant challenges. We report a 53-year-old female diagnosed with a...

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Main Authors: Wenguang He, Congcong Song, Jiwei Ren, Xiao Ji, Xiuyan Wang, Lixia Liu
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-04-01
Series:Frontiers in Pharmacology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fphar.2024.1336699/full
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author Wenguang He
Congcong Song
Jiwei Ren
Xiao Ji
Xiuyan Wang
Lixia Liu
author_facet Wenguang He
Congcong Song
Jiwei Ren
Xiao Ji
Xiuyan Wang
Lixia Liu
author_sort Wenguang He
collection DOAJ
description Cholangiocarcinoma (CCA) is a highly heterogeneous tumor that occurs in the bile duct epithelium; adenosquamous carcinoma is a rare pathological subtype of CCA. The clinical treatment of patients with metastatic distal CCA poses significant challenges. We report a 53-year-old female diagnosed with a stage III adenosquamous carcinomas of distal CCA. Metastasis occurred 4 months postoperatively and she was diagnosed with stage IV disease. The patient was treated with Gemcitabine + Oxaliplatin (GEMOX) and Capecitabine + Oxaliplatin (CAPEOX), followed by sintilimab monotherapy. After two cycles of treatment, the patient achieved partial response (PR) and the lesion continued to shrink. After 37 months of follow-up, the patient’s liver metastasis had almost completely disappeared, and complete response (CR) was achieved. Moreover, she had more than 46 months of disease progression-free survival (PFS). Immunohistochemical testing showed high expression of PD-L1, and next-generation sequencing revealed the presence of mutations in DNA damage repair (DDR) pathway genes. To the best of our knowledge, this is the first reported case of the successful treatment of metastatic distal adenosquamous CCA with sintilimab alone. Remarkably, patients of CCA with high PD-L1 expression and DDR pathway gene mutations may benefit from sintilimab treatment.
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spelling doaj.art-a218675e3fbd445b803e49e543b83fca2024-04-10T04:28:15ZengFrontiers Media S.A.Frontiers in Pharmacology1663-98122024-04-011510.3389/fphar.2024.13366991336699Case report: Robust response to sintilimab in advanced distal cholangiocarcinoma with PD-L1 expression and DNA damage repairWenguang He0Congcong Song1Jiwei Ren2Xiao Ji3Xiuyan Wang4Lixia Liu5Traditional Chinese Medicine Department, Shanxi Province Cancer Hospital, Shanxi Hospital Affiliated to Cancer Hospital Chinese Academy of Medical Sciences, Cancer Hospital Affiliated to Shanxi Medical University, Taiyuan, ChinaShenzhen Engineering Center for Translational Medicine of Precision Cancer Immunodiagnosis and Therapy, YuceBio Technology Co., Ltd., Shenzhen, ChinaTraditional Chinese Medicine Department, Shanxi Province Cancer Hospital, Shanxi Hospital Affiliated to Cancer Hospital Chinese Academy of Medical Sciences, Cancer Hospital Affiliated to Shanxi Medical University, Taiyuan, ChinaTraditional Chinese Medicine Department, Shanxi Province Cancer Hospital, Shanxi Hospital Affiliated to Cancer Hospital Chinese Academy of Medical Sciences, Cancer Hospital Affiliated to Shanxi Medical University, Taiyuan, ChinaShenzhen Engineering Center for Translational Medicine of Precision Cancer Immunodiagnosis and Therapy, YuceBio Technology Co., Ltd., Shenzhen, ChinaTraditional Chinese Medicine Department, Shanxi Province Cancer Hospital, Shanxi Hospital Affiliated to Cancer Hospital Chinese Academy of Medical Sciences, Cancer Hospital Affiliated to Shanxi Medical University, Taiyuan, ChinaCholangiocarcinoma (CCA) is a highly heterogeneous tumor that occurs in the bile duct epithelium; adenosquamous carcinoma is a rare pathological subtype of CCA. The clinical treatment of patients with metastatic distal CCA poses significant challenges. We report a 53-year-old female diagnosed with a stage III adenosquamous carcinomas of distal CCA. Metastasis occurred 4 months postoperatively and she was diagnosed with stage IV disease. The patient was treated with Gemcitabine + Oxaliplatin (GEMOX) and Capecitabine + Oxaliplatin (CAPEOX), followed by sintilimab monotherapy. After two cycles of treatment, the patient achieved partial response (PR) and the lesion continued to shrink. After 37 months of follow-up, the patient’s liver metastasis had almost completely disappeared, and complete response (CR) was achieved. Moreover, she had more than 46 months of disease progression-free survival (PFS). Immunohistochemical testing showed high expression of PD-L1, and next-generation sequencing revealed the presence of mutations in DNA damage repair (DDR) pathway genes. To the best of our knowledge, this is the first reported case of the successful treatment of metastatic distal adenosquamous CCA with sintilimab alone. Remarkably, patients of CCA with high PD-L1 expression and DDR pathway gene mutations may benefit from sintilimab treatment.https://www.frontiersin.org/articles/10.3389/fphar.2024.1336699/fullcholangiocarcinomasintilimabPD-L1DNA damage repaircomplete response
spellingShingle Wenguang He
Congcong Song
Jiwei Ren
Xiao Ji
Xiuyan Wang
Lixia Liu
Case report: Robust response to sintilimab in advanced distal cholangiocarcinoma with PD-L1 expression and DNA damage repair
Frontiers in Pharmacology
cholangiocarcinoma
sintilimab
PD-L1
DNA damage repair
complete response
title Case report: Robust response to sintilimab in advanced distal cholangiocarcinoma with PD-L1 expression and DNA damage repair
title_full Case report: Robust response to sintilimab in advanced distal cholangiocarcinoma with PD-L1 expression and DNA damage repair
title_fullStr Case report: Robust response to sintilimab in advanced distal cholangiocarcinoma with PD-L1 expression and DNA damage repair
title_full_unstemmed Case report: Robust response to sintilimab in advanced distal cholangiocarcinoma with PD-L1 expression and DNA damage repair
title_short Case report: Robust response to sintilimab in advanced distal cholangiocarcinoma with PD-L1 expression and DNA damage repair
title_sort case report robust response to sintilimab in advanced distal cholangiocarcinoma with pd l1 expression and dna damage repair
topic cholangiocarcinoma
sintilimab
PD-L1
DNA damage repair
complete response
url https://www.frontiersin.org/articles/10.3389/fphar.2024.1336699/full
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