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Case Reports
. 2023 Apr 28;12(4):1041-1048.
doi: 10.21037/tcr-22-1679. Epub 2023 Mar 16.

Effectiveness of tislelizumab when combined with etoposide and carboplatin in patients with SMARCA4-deficient undifferentiated thoracic tumor: a case report

Affiliations
Case Reports

Effectiveness of tislelizumab when combined with etoposide and carboplatin in patients with SMARCA4-deficient undifferentiated thoracic tumor: a case report

Pan Yang et al. Transl Cancer Res. .

Abstract

Background: SMARCA4-deficient undifferentiated thoracic tumor (SMARCA4-UT) is a rare malignant condition with high invasiveness and poor prognosis. Presently, no clear guidelines are available for the treatment of SMARCA4-UT. The median overall survival was only four to seven months. Several patients are diagnosed in advanced stages of the malignancy and do not respond to conventional radiotherapy and chemotherapy.

Case description: A 51-year-old Chinese man was diagnosed with a SMARCA4-UT. The patient did not have a chronic history of hypertension or diabetes and any family history of malignant tumors. No sensitive mutation in lung cancer-related ten genes was identified. The first-line therapy with four cycles of liposomal paclitaxel and cisplatin combined with two cycles of tyrosine kinase inhibitor anlotinib failed. On immunohistochemical analysis, no programmed cell death 1 ligand 1 (PD-L1) expression was found. However, whole-exon sequencing revealed a high tumor mutation burden (TMB) of 15.95 mutations/Mb with TP53 and KEAP1 mutations. The patient was treated with a second-line regimen containing tislelizumab, etoposide, and carboplatin (TEC). A reduction in tumor burden was seen for more than 10 months.

Conclusions: The case of SMARCA4-UT with a high mutation burden successfully responded to the combined regimen containing TEC. This could be a new treatment option for patients with SMARCA4-UTs.

Keywords: SMARCA4-deficient undifferentiated thoracic tumor (SMARCA4-UT); Tislelizumab; immunotherapy; short-term curative effect.

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Conflict of interest statement

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tcr.amegroups.com/article/view/10.21037/tcr-22-1679/coif). The authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Chest computed tomography images: (A) Baseline before treatment; (B) progressive disease after four cycles of chemotherapy and 2 cycles of anlotinib; (C) partial response after two doses of tislelizumab, etoposide, and carboplatin; (D) after five doses of tislelizumab, etoposide, and carboplatin.
Figure 2
Figure 2
Histomorphology and immunohistochemical features of thoracic SMARCA4-UT. (A) Hematoxylin and eosin staining (×200). (B) SMARCA4 staining (×200). (C) SALL4 staining (×200). (D) SOX-2 staining (×200). (E) CD34 staining (×200). (F) PD-L1 staining (×200). PD-L1, programmed cell death ligand 1; SMARCA4-UT, SMARCA4-deficient undifferentiated thoracic tumor.

References

    1. Arnaud O, Le Loarer F, Tirode F. BAFfling pathologies: Alterations of BAF complexes in cancer. Cancer Lett 2018;419:266-79. 10.1016/j.canlet.2018.01.046 - DOI - PubMed
    1. Nambirajan A, Singh V, Bhardwaj N, et al. SMARCA4/BRG1-Deficient Non-Small Cell Lung Carcinomas: A Case Series and Review of the Literature. Arch Pathol Lab Med 2021;145:90-8. 10.5858/arpa.2019-0633-OA - DOI - PubMed
    1. Yoshida A, Kobayashi E, Kubo T, et al. Clinicopathological and molecular characterization of SMARCA4-deficient thoracic sarcomas with comparison to potentially related entities. Mod Pathol 2017;30:797-809. 10.1038/modpathol.2017.11 - DOI - PubMed
    1. Yoshida A, Boland JM, Loarer FL, et al. Thoracic SMARCA4-deficient undifferentiated tumor. WHO Classification of Tumours Editorial Board. Thoracic Tumours. WHO classification of tumours series, 5th edition; vol. 5. Lyon, France: International Agency for Research on Cancer; 2021. [cited 2021 May 27].
    1. Perret R, Chalabreysse L, Watson S, et al. SMARCA4-deficient Thoracic Sarcomas: Clinicopathologic Study of 30 Cases With an Emphasis on Their Nosology and Differential Diagnoses. Am J Surg Pathol 2019;43:455-65. 10.1097/PAS.0000000000001188 - DOI - PubMed

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