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SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a new clinical entity characterized by SMARCA4 inactivation and has a dismal prognosis because of rapid growth. Effective treatments for SMARCA4-DTS have not yet been developed. Most recently, anti-programmed cell death 1 receptor (PD-1) blockade has been effective for SMARCA4-deficient lung cancer and malignant rhabdoid tumor-like tumors. Here, we describe a patient with SMARCA4-DTC who experienced a marked response to the administration of pembrolizumab. A 70-year-old female was referred to our department for treatment of SMARCA4-DTC. Positron emission tomography-computed tomography had revealed a left mediastinal tumor, peritoneal dissemination and multiple cutaneous metastases at diagnosis. Immunohistochemical analyses revealed 60% of tumor cells expressed programmed cell death ligand 1 (PD-L1). The patient was given pembrolizumab as first-line treatment. Pembrolizumab suppressed tumor growth dramatically, with only one dose leading to a partial response. Our case suggests the immunohistochemical analysis of PD-L1 expression be undertaken for patients with SMARCA4-DTS and that pembrolizumab treatment may be a promising strategy for PD-L1-positive SMARCA4-DTS.

Titel:	Exceptionally rapid response to pembrolizumab in a SMARCA4-deficient thoracic sarcoma overexpressing PD-L1: A case report.
Autor/in / Beteiligte Person:	Takada, K ; Sugita, S ; Murase, K ; Kikuchi, T ; Oomori, G ; Ito, R ; Hayasaka, N ; Miyanishi, K ; Iyama, S ; Ikeda, H ; Kobune, M ; Emori, M ; Kato, J ; Hasegawa, T
Link:	Full Text Finder (Volltext)
Zeitschrift:	Thoracic cancer, Jg. 10 (2019-12-01), Heft 12, S. 2312-2315
Veröffentlichung:	November 2012- : Singapore : Tianjin : Wiley Publishing Asia Pty Ltd ; Tianjin Lung Cancer Institute ; <i>Original Publication</i>: Richmond, Vic. : Tianjin : Blackwell Pub. Asia Pty Ltd. ; Tianjin Lung Cancer Institute, 2019
Medientyp:	report
ISSN:	1759-7714 (electronic)
DOI:	10.1111/1759-7714.13215
Schlagwort:	Aged Antibodies, Monoclonal, Humanized administration & dosage Antibodies, Monoclonal, Humanized adverse effects Antineoplastic Agents, Immunological administration & dosage Antineoplastic Agents, Immunological adverse effects Biomarkers, Tumor Female Humans Immunohistochemistry Molecular Targeted Therapy Positron Emission Tomography Computed Tomography Thoracic Neoplasms diagnosis Treatment Outcome Antibodies, Monoclonal, Humanized therapeutic use Antineoplastic Agents, Immunological therapeutic use B7-H1 Antigen antagonists & inhibitors DNA Helicases deficiency Gene Expression Nuclear Proteins deficiency Thoracic Neoplasms drug therapy Thoracic Neoplasms etiology Transcription Factors deficiency
Sonstiges:	Nachgewiesen in: MEDLINE Sprachen: English Publication Type: Case Reports Language: English [Thorac Cancer] 2019 Dec; Vol. 10 (12), pp. 2312-2315. <i>Date of Electronic Publication: </i>2019 Oct 16. MeSH Terms: Gene Expression* ; Antibodies, Monoclonal, Humanized / therapeutic use ; Antineoplastic Agents, Immunological / therapeutic use ; B7-H1 Antigen / antagonists & inhibitors ; DNA Helicases / deficiency ; Nuclear Proteins / deficiency ; Thoracic Neoplasms / drug therapy ; Thoracic Neoplasms / etiology ; Transcription Factors / deficiency ; Aged ; Antibodies, Monoclonal, Humanized / administration & dosage ; Antibodies, Monoclonal, Humanized / adverse effects ; Antineoplastic Agents, Immunological / administration & dosage ; Antineoplastic Agents, Immunological / adverse effects ; Biomarkers, Tumor ; Female ; Humans ; Immunohistochemistry ; Molecular Targeted Therapy ; Positron Emission Tomography Computed Tomography ; Thoracic Neoplasms / diagnosis ; Treatment Outcome Comments: Comment in: Thorac Cancer. 2021 Jan;12(1):140. (PMID: 33075212) ; Comment in: Thorac Cancer. 2021 Jan;12(1):141. (PMID: 33210422) ; Erratum in: Thorac Cancer. 2020 Dec;11(12):3645. (PMID: 33258562) References: Science. 2018 Feb 16;359(6377):770-775. (PMID: 29301958) ; Ann Oncol. 2019 Jul 1;30(7):1096-1103. (PMID: 31038663) ; Ann Oncol. 2019 Aug 1;30(8):1401-1403. (PMID: 31114851) ; Thorac Cancer. 2019 May;10(5):1285-1288. (PMID: 30972962) ; N Engl J Med. 2016 Nov 10;375(19):1823-1833. (PMID: 27718847) ; Nat Genet. 2015 Oct;47(10):1200-5. (PMID: 26343384) ; Am J Surg Pathol. 2019 Apr;43(4):455-465. (PMID: 30451731) ; Lancet Oncol. 2017 Sep;18(9):1182-1191. (PMID: 28734759) ; Science. 2018 Feb 16;359(6377):801-806. (PMID: 29301960) ; J Clin Invest. 2017 Aug 1;127(8):2930-2940. (PMID: 28650338) ; Sci Adv. 2015 Jun 12;1(5):e1500447. (PMID: 26601204) ; Nature. 2014 Nov 27;515(7528):568-71. (PMID: 25428505) ; J Natl Cancer Inst. 2018 Jul 1;110(7):787-790. (PMID: 29365144) ; Nature. 1998 Jul 9;394(6689):203-6. (PMID: 9671307) Contributed Indexing: Keywords: PD-L1; SMARCA4-deficient thoracic sarcoma; pembrolizumab Substance Nomenclature: 0 (Antibodies, Monoclonal, Humanized) ; 0 (Antineoplastic Agents, Immunological) ; 0 (B7-H1 Antigen) ; 0 (Biomarkers, Tumor) ; 0 (CD274 protein, human) ; 0 (Nuclear Proteins) ; 0 (Transcription Factors) ; DPT0O3T46P (pembrolizumab) ; EC 3.6.1.- (SMARCA4 protein, human) ; EC 3.6.4.- (DNA Helicases) Entry Date(s): Date Created: 20191017 Date Completed: 20200810 Latest Revision: 20210428 Update Code: 20231215 PubMed Central ID: PMC6885443

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Exceptionally rapid response to pembrolizumab in a SMARCA4-deficient thoracic sarcoma overexpressing PD-L1: A case report.