Wu C.-W.CHING-YAO YANGYIH-LEONG CHANGJIN-YUAN SHIH2021-05-022021-05-0220201178-6930https://www.scopus.com/inward/record.uri?eid=2-s2.0-85092347642&doi=10.2147%2fOTT.S262653&partnerID=40&md5=10d3242802484a967bb213d455230a67https://scholars.lib.ntu.edu.tw/handle/123456789/558534Pulmonary pleomorphic carcinoma (PPC) generally lacks actionable driver mutations such as epidermal growth factor receptor mutations or anaplastic lymphoma kinase or c-ros oncogene 1 (ROS1) rearrangements. The response to crizotinib, ceritinib, brigatinib, and lorlatinib in ROS1-positive advanced non-small cell lung carcinoma is well established; however, there is little mention of their successful administration in pulmonary pleomorphic carcinoma cases. We report a case of a stage II PPC with recurrence after surgical resection and developed multiple distant metastasis. The tumor was refractory to chemotherapy and immunotherapy with progressive disease. EZR-ROS1 fusion was detected by next-generation sequencing and showed a good response to serial ROS1 inhibitors combined with surgery and radiotherapy. Now under lorlatinib, all her lesions responded well during the follow-up with sustained partial remission for more than 18 months. A sustainable treatment effect can be achieved in pulmonary pleomorphic carcinoma with driver mutations with tyrosine kinase inhibitor treatment. Driver mutations should be regularly tested in pulmonary pleomorphic carcinomas. ? 2020 Wu et al.[SDGs]SDG3alanine aminotransferase; aspartate aminotransferase; cisplatin; crizotinib; docetaxel; homeobox protein Nkx 2.1; lorlatinib; pembrolizumab; pemetrexed; vimentin; adult; Article; bone metastasis; brain metastasis; cancer adjuvant therapy; cancer radiotherapy; cancer recurrence; cancer staging; cancer surgery; cancer therapy; case report; clavicle; clinical article; computer assisted tomography; drug substitution; drug withdrawal; EZR ROS1 fusion gene; female; femur; follow up; frontal lobe; fusion gene; gene; gene rearrangement; high throughput sequencing; human; human tissue; ischium; kidney metastasis; liver toxicity; lung carcinoma; lung lobe; lung lobectomy; middle aged; multiple cycle treatment; non-smoker; positron emission tomography; protein expression; pulmonary pleomorphic carcinoma; rib; ROS1 gene; salvage therapyjournal article10.2147/OTT.S2626532-s2.0-85092347642