{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T06:50:43Z","timestamp":1791442243043,"version":"4.3.3"},"reference-count":13,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T00:00:00Z","timestamp":1791331200000},"content-version":"vor","delay-in-days":6,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100002560","name":"Soonchunhyang University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002560","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pathology International"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms characterized by melanocytic and smooth muscle differentiation, commonly associated with\n                    <jats:italic>TSC1\/TSC2<\/jats:italic>\n                    alterations or\n                    <jats:italic>TFE3<\/jats:italic>\n                    rearrangements. Herein, we report a rare case of urinary bladder PEComa with MITF overexpression and without identifiable\n                    <jats:italic>TSC1\/TSC2<\/jats:italic>\n                    mutations or\n                    <jats:italic>TFE3<\/jats:italic>\n                    rearrangement. A 50\u2010year\u2010old man underwent partial cystectomy for a bladder mass. Histologically, the tumor was well circumscribed and composed of mixed spindle and epithelioid cells with cytologic atypia and low mitotic activity. Immunohistochemistry revealed diffuse expression of SMA, HMB45, and MITF, with negative staining for TFE3. Quantitative reverse\u2010transcription PCR revealed increased MITF mRNA expression in PEComa tissue. Next\u2010generation sequencing identified a truncating\n                    <jats:italic>ATRX<\/jats:italic>\n                    mutation but no alterations in\n                    <jats:italic>TSC1\/TSC2<\/jats:italic>\n                    or\n                    <jats:italic>TFE3<\/jats:italic>\n                    , and no\n                    <jats:italic>MITF<\/jats:italic>\n                    copy number gain. By Folpe criteria, the tumor was classified as malignant, whereas alternative models suggested a lower\u2010risk category, highlighting the limitations of histologic risk stratification systems. The absence of canonical\n                    <jats:italic>TSC\/mTOR<\/jats:italic>\n                    or\n                    <jats:italic>TFE3<\/jats:italic>\n                    alterations, together with MITF overexpression, suggests a distinct molecular mechanism potentially involving dysregulation of the MiT\/TFE pathway. This case expands the molecular spectrum of PEComa and supports the existence of a MITF\u2010driven subset, highlighting the need for integrated histologic and molecular evaluation for prognostic assessment.\n                  <\/jats:p>","DOI":"10.1111\/pin.70187","type":"journal-article","created":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T06:10:12Z","timestamp":1791439812000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["MITF\u2010Overexpressing Perivascular Epithelioid Cell Tumor of the Urinary Bladder Lacking TSC1\/TSC2 Alterations and TFE3 Rearrangement: A Case Report and Review of the Literature"],"prefix":"10.1111","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-7538-5296","authenticated-orcid":false,"given":"Ji\u2010In","family":"Jeong","sequence":"first","affiliation":[{"name":"Departments of Pathology, College of Medicine Soonchunhyang University Cheonan Hospital Cheonan Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-4549-633X","authenticated-orcid":false,"given":"Jiwoo","family":"Lee","sequence":"additional","affiliation":[{"name":"Departments of Pathology, College of Medicine Soonchunhyang University Cheonan Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mee\u2010Hye","family":"Oh","sequence":"additional","affiliation":[{"name":"Departments of Pathology, College of Medicine Soonchunhyang University Cheonan Hospital Cheonan Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyun Deuk","family":"Cho","sequence":"additional","affiliation":[{"name":"Departments of Pathology, College of Medicine Soonchunhyang University Cheonan Hospital Cheonan Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji\u2010Hye","family":"Lee","sequence":"additional","affiliation":[{"name":"Departments of Pathology, College of Medicine Soonchunhyang University Cheonan Hospital Cheonan Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Youn Soo","family":"Jeon","sequence":"additional","affiliation":[{"name":"Departments of Urology, College of Medicine Soonchunhyang University Cheonan Hospital Cheonan Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7605-086X","authenticated-orcid":false,"given":"Si\u2010Hyong","family":"Jang","sequence":"additional","affiliation":[{"name":"Departments of Pathology, College of Medicine Soonchunhyang University Cheonan Hospital Cheonan Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,10,7]]},"reference":[{"issue":"7","key":"e_1_2_10_2_1","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1093\/jjco\/hyaf056","article-title":"PEComa\u2010Its Clinical Features, Histopathology, and Current Therapy","volume":"55","author":"Izubuchi Y.","year":"2025","journal-title":"Japanese Journal of Clinical Oncology"},{"issue":"1","key":"e_1_2_10_3_1","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1093\/ajcp\/aqaa204","article-title":"Perivascular Epithelioid Cell Tumor of Urinary Bladder","volume":"156","author":"Wu Y.","year":"2020","journal-title":"American Journal of Clinical Pathology"},{"issue":"3","key":"e_1_2_10_4_1","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.afju.2018.04.005","article-title":"Bladder Perivascular Epithelioid Cell Tumors","volume":"24","author":"Tricard T.","year":"2018","journal-title":"African Journal of Urology"},{"issue":"2","key":"e_1_2_10_5_1","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1053\/j.semdp.2015.02.006","article-title":"PEComas of the Kidney and of the Genitourinary Tract","volume":"32","author":"Martignoni G.","year":"2015","journal-title":"Seminars in Diagnostic Pathology"},{"issue":"12","key":"e_1_2_10_6_1","doi-asserted-by":"crossref","first-page":"1558","DOI":"10.1097\/01.pas.0000173232.22117.37","article-title":"Perivascular Epithelioid Cell Neoplasms of Soft Tissue and Gynecologic Origin: A Clinicopathologic Study of 26 Cases and Review of the Literature","volume":"29","author":"Folpe A. L.","year":"2005","journal-title":"American Journal of Surgical Pathology"},{"issue":"11","key":"e_1_2_10_7_1","doi-asserted-by":"crossref","first-page":"1381","DOI":"10.1097\/PAS.0000000000002276","article-title":"PEComa With MITF Overexpression: Clinicopathologic and Molecular Analysis of a Series of 36 Cases","volume":"48","author":"Hanna J.","year":"2024","journal-title":"American Journal of Surgical Pathology"},{"key":"e_1_2_10_8_1","first-page":"1","article-title":"Perivascular Epithelioid Cell Tumors of the Urinary Bladder: A Multi\u2010Institutional Clinicopathologic and Molecular Analysis of 21 Cases","author":"Sangoi A. R.","year":"2025","journal-title":"Virchows Archiv"},{"issue":"5","key":"e_1_2_10_9_1","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1097\/00000478-200305000-00013","article-title":"Clear Cell Myomelanocytic Tumor of the Urinary Bladder","volume":"27","author":"Pan C. C.","year":"2003","journal-title":"American Journal of Surgical Pathology"},{"issue":"2","key":"e_1_2_10_10_1","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1097\/PAS.0000000000001592","article-title":"Histopathologic Characterization of Bladder Perivascular Epithelioid Cell Neoplasms (PEComa): A Series of 11 Cases With a Subset Having TFE3 Rearrangements","volume":"45","author":"Neumann N. M.","year":"2021","journal-title":"American Journal of Surgical Pathology"},{"issue":"2","key":"e_1_2_10_11_1","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1097\/PAS.0b013e3181854929","article-title":"Perivascular Epithelioid Cell Tumor (PEComa) of the Urinary Bladder: Report of 3 Cases and Review of the Literature","volume":"33","author":"Sukov W. R.","year":"2009","journal-title":"American Journal of Surgical Pathology"},{"issue":"5","key":"e_1_2_10_12_1","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1097\/PAS.0b013e3181d90370","article-title":"Renal Epithelioid Angiomyolipoma With Atypia: A Series of 40 Cases With Emphasis on Clinicopathologic Prognostic Indicators of Malignancy","volume":"34","author":"Brimo F.","year":"2010","journal-title":"American Journal of Surgical Pathology"},{"issue":"3","key":"e_1_2_10_13_1","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1111\/his.15476","article-title":"Genetic Correlates of Malignancy in TFE3\u2010Rearranged PEComa: A Series of 14 Cases","volume":"87","author":"Papke D. J.","year":"2025","journal-title":"Histopathology"},{"issue":"3","key":"e_1_2_10_14_1","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1007\/s00428-024-03855-z","article-title":"Renal Epithelioid Angiomyolipomas Overexpress TFE3 and the TFE3\u2010Regulated Gene TRIM63 in the Absence of TFE3 Rearrangement","volume":"485","author":"Collins K.","year":"2024","journal-title":"Virchows Archiv: An International Journal of Pathology"}],"container-title":["Pathology International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pin.70187","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/pin.70187","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pin.70187","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T06:10:18Z","timestamp":1791439818000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pin.70187"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10]]},"references-count":13,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2026,10]]}},"alternative-id":["10.1111\/pin.70187"],"URL":"https:\/\/doi.org\/10.1111\/pin.70187","archive":["Portico"],"relation":{},"ISSN":["1320-5463","1440-1827"],"issn-type":[{"value":"1320-5463","type":"print"},{"value":"1440-1827","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10]]},"assertion":[{"value":"2026-06-09","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-21","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-10-07","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70187"}}