Case reportFirst-line crizotinib therapy is effective for a novel SEC31A-anaplastic lymphoma kinase fusion in a patient with stage IV lung adenocarcinoma: a case report and literature reviewsWu, Rongronga,*; Liu, Shinanb,*; Lv, Guolib; Deng, Chaowenb; Wang, Ruolanb; Zhang, Shenglinb; Zhu, Dongyib; Wang, Leb; Lei, Youmingc; Luo, Zhuangb Author Information aDepartment of Radiology, The First People’s Hospital of Yunnan Province/Affiliated Hospital of Kunming University of Science and Technology and Departments of bRespiratory and Critical Care Medicine cGeriatric Thoracic Surgery, The First Affiliated Hospital of Kunming Medical University, Kunming, China Received 26 March 2022 Revised form accepted 28 March 2022 *Rongrong Wu and Shinan Liu contributed equally to the writing of this article. Supplemental Digital Content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's website, www.anti-cancerdrugs.com. Correspondence to Dr. Zhuang Luo, Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Kunming Medical University, No. 295 Xichang Road,Wuhua District, Kunming, Yunnan, 650032, China, Tel/fax: +86 0871 65324888 2512; e-mail: [email protected] Anti-Cancer Drugs ():10.1097/CAD.0000000000001408, November 16, 2022. | DOI: 10.1097/CAD.0000000000001408 Buy SDC PAP Metrics Abstract Anaplastic lymphoma kinase (ALK) fusion was found in 3–7% of all patients with nonsmall cell lung cancer. The efficacy of ALK-tyrosine kinase inhibitor (ALK-TKI) in EML4-ALK has been extensively studied, whereas little evidence is available on its efficacy in rare ALK fusions. Here, we report the performance of crizotinib in a 50-year-old male lung adenocarcinoma patient with a novel rare SEC31A-ALK fusion. Computed tomography (CT) scan revealed multiple patchy high-density shadows in both lungs. The larger ones are located near the spine in the right lung lower lobe (55 × 34 mm) and the left hilar region (45 × 26 mm), with multiple enlarged mediastinal and axillary lymph nodes. Biopsy by bronchoscopy revealed invasive adenocarcinoma. The pathological stage of T4N3M1b (clinical stage: IVA) was confirmed. Next-generation sequencing revealed SEC31A: exon20~ALK: exon20 fusion, ABCB1 amplification, FGF19 amplification, DAXX p.S213L, MUTYH p.R19*(germline mutation and pathogenic) with tumor mutational burden at 3.2 mutations/Mb, microsatellite stable, proficient mismatch repair and PD-L1 positive [immunohistochemistry, tumor proportion score(TPS) 1–49% (TPS = 25%)]. Based on these findings, crizotinib was recommended for the first-line treatment at 250 mg twice daily. The first CT assessment after 2-month therapy showed partial response (PR) for the two larger lesions, multiple shadows and nodules in both lungs and the mediastinal and axillary lymph nodes. Crizotinib at 250 mg twice a day was applied in the following 9 months. Assessment at every 3 months (up to 1-year after diagnosis) showed further absorption for all lesions (continuous PR). We reported a novel rare ALK fusion SEC31A: EXON20~ALK: exon20 and showed the effectiveness of crizotinib against the fusion. This study provided strong evidence for the efficacy of ALK-TKI for rare ALK fusion. Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.