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We report a case of renal medullary carcinoma, immunohistochemically defined as SMARCB1 deficient, in a 67\u2010year\u2010old Japanese woman without a history of sickle cell trait. Somatic mutation of <jats:italic>SMARCB1<\/jats:italic> and an <jats:italic>EML4<\/jats:italic>::<jats:italic>ALK<\/jats:italic> fusion gene were identified by comprehensive genomic profiling. Computed tomography revealed metastatic lesions in the retrocaval lymph nodes, liver, and bronchus. Six cycles of the dose\u2010dense methotrexate, vinblastine, adriamycin, and cisplatin\u2010combined chemotherapy were completed after an ultrasound\u2010guided percutaneous biopsy of the renal tumor. After chemotherapy, the size of the original tumor in the right kidney had decreased in size, as well as the other metastatic lesions.<\/jats:p>","DOI":"10.1111\/pin.13494","type":"journal-article","created":{"date-parts":[[2024,11,6]],"date-time":"2024-11-06T10:45:39Z","timestamp":1730889939000},"page":"704-707","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["SMARCB1\u2010deficient renal medullary carcinoma with an <i>EML4<\/i>::<i>ALK<\/i> fusion gene in a Japanese woman"],"prefix":"10.1111","volume":"74","author":[{"given":"Megumi","family":"Nobuoka","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine Shinshu University Hospital Matsumoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsuya","family":"Mukawa","sequence":"additional","affiliation":[{"name":"Department of Urology Shinshu University School of Medicine Matsumoto 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