Cancer Res Treat.  2024 Jul;56(3):967-971. 10.4143/crt.2023.1308.

Molecular and Treatment Characteristics of SMARCB1 or SMARCA4-Deficient Undifferentiated Tumor: Retrospective Case Series

• Kang HG
• Koh J
• Kim TM
• Han DH
• Won TB
• Kim DW
• Kim DY
• Keam B

• KMID: 2557683
• DOI: http://doi.org/10.4143/crt.2023.1308

Abstract

SMARCB1 or SMARCA4-deficient sinonasal carcinoma or thoracic undifferentiated tumor has aggressive nature with a poor prognosis. Patients with this disease were diagnosed by immunohistochemistry or next-generation sequencing. Those who were able to receive a surgery tended to be cured, while the others treated with chemotherapy, radiation therapy, or immune checkpoint inhibitor were often insensitive to these therapies. However, one having CD274 (PD-L1) amplification showed the response to immune checkpoint inhibitor and a good prognosis. We believed that this report could provide promising information for determining the optimal treatment option.

Keyword

SMARCB1; SMARCA4; Undifferentiated tumor

Figure

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  Fig. 1. Clinical response to durvalumab in CD274-amplified patient with SMARCA4-deficient thoracic undifferentiated tumor. Initial computed tomography (CT) image before therapy (A). CT image after concurrent chemoradiation (B). Follow-up CT image after treating with consolidation durvalumab 13 cycles (C).

Reference

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