{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T07:04:03Z","timestamp":1785135843704,"version":"3.55.0"},"reference-count":14,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,6,21]],"date-time":"2026-06-21T00:00:00Z","timestamp":1782000000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,6,21]],"date-time":"2026-06-21T00:00:00Z","timestamp":1782000000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["IJU Case Reports"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>\n                      Epithelioid angiomyolipoma is a rare perivascular epithelioid cell tumor subtype with malignant potential. Its morphology mimics renal cell carcinoma, posing diagnostic challenges. Half of these cases harbor\n                      <jats:italic>TSC1<\/jats:italic>\n                      \/\n                      <jats:italic>TSC2<\/jats:italic>\n                      alterations, and a small subset features\n                      <jats:italic>TFE3<\/jats:italic>\n                      rearrangements, mutually exclusive of\n                      <jats:italic>TSC<\/jats:italic>\n                      mutations. Recently, \u201cperivascular epithelioid cell tumor\u2010like neoplasms\u201d with\n                      <jats:italic>ASPSCR1::TFE3<\/jats:italic>\n                      fusion lacking melanocytic markers were described.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation<\/jats:title>\n                    <jats:p>\n                      A 25\u2010year\u2010old woman presented with a 4.5\u2010cm renal mass. Biopsy suggested\n                      <jats:italic>TFE3<\/jats:italic>\n                      \u2010rearranged renal cell carcinoma because of diffuse TFE3 positivity and no HMB45 immunoreactivity. Postoperatively, despite a complete lack of melanocytic markers, the tumor was diagnosed as\n                      <jats:italic>TFE3<\/jats:italic>\n                      \u2010rearranged epithelioid angiomyolipoma based on its epithelioid morphology and PAX8 negativity, including a retrospective biopsy study. Fluorescence in\u00a0situ hybridization confirmed\n                      <jats:italic>ASPSCR1::TFE3<\/jats:italic>\n                      fusion, suggesting this rare, newly recognized entity.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      <jats:italic>TFE3<\/jats:italic>\n                      \u2010rearranged epithelioid angiomyolipoma can lack melanocytic markers. PAX8 immunohistochemistry and genetic testing help distinguish this potentially aggressive tumor from renal cell carcinoma and\n                      <jats:italic>TSC<\/jats:italic>\n                      \u2010mutated subtypes.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/iju5.70213","type":"journal-article","created":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T05:46:14Z","timestamp":1782107174000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["<i>ASPSCR1: TFE3<\/i>\n                    Fusion\u2010Positive Epithelioid Angiomyolipoma\/PEComa Lacking Melanocytic Markers: A Case Report"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-9419-3190","authenticated-orcid":false,"given":"Yuka","family":"Kondo","sequence":"first","affiliation":[{"name":"Department of Diagnostic Pathology, School of Medicine Fujita Health University  Toyoake Aichi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6536-648X","authenticated-orcid":false,"given":"Yoji","family":"Nagashima","sequence":"additional","affiliation":[{"name":"Department of Surgical Pathology Tokyo Women's Medical University Hospital  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8455-7364","authenticated-orcid":false,"given":"Kenji","family":"Zennami","sequence":"additional","affiliation":[{"name":"Department of Urology Nagoya University Graduate School of Medicine  Nagoya Aichi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-7127-2163","authenticated-orcid":false,"given":"Megumi","family":"Sato","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology, School of Medicine Fujita Health University  Toyoake Aichi 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