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Although most behave in an indolent fashion, a subset may develop metastasis. Herein, we describe the clinicopathologic and molecular characteristics of 21 bladder PEComas, including biomarker analysis and comprehensive sequencing. Patients included 13 females and 8 males, with age ranging from 17\u201381\u00a0years (mean\u2009=\u200947.6\u00a0years). Clinical follow-up data was available for 17 patients (ranging 5\u201360\u00a0months; mean\u2009=\u200919.4\u00a0months). The morphologic features significantly associated with metastatic disease included\u2009\u2265\u20092 mitoses\/10 high-power fields (p\u2009=\u20090.0023), atypical mitoses (p\u2009=\u20090.0152), and necrosis (p\u2009=\u20090.0023); the presence of\u2009\u2265\u200970% atypical epithelioid cells and vascular invasion did not meet statistical significance. The Biomarker profile (p16, p53, TRIM63 ISH, ATRX, RB1) found no statistical significance with metastasis. TRIM63 ISH showed high sensitivity (86%)\u00a0with poor specificity (11%) for\u00a0\n                    <jats:italic>TFE3<\/jats:italic>\n                    \u00a0rearrangements. NGS revealed\n                    <jats:italic>TFE3<\/jats:italic>\n                    fusions in 8\/17 cases (47%): 7 with\n                    <jats:italic>SFPQ::TFE3<\/jats:italic>\n                    \u00a0fusions and 1 with\n                    <jats:italic>NONO::TFE3<\/jats:italic>\n                    fusion). Overall, mTOR pathway mutations were detected in 9 cases (53%):\n                    <jats:italic>TSC1\/2<\/jats:italic>\n                    \u00a0mutations in 6 (35%),\u00a0\n                    <jats:italic>MTOR<\/jats:italic>\n                    \u00a0mutation in 1 (6%),\u00a0and co-mutations of\u00a0\n                    <jats:italic>TSC\/MTOR<\/jats:italic>\n                    \u00a0in 2 (12%) cases. Additionally, co-mutations involving\u00a0\n                    <jats:italic>p53<\/jats:italic>\n                    \u00a0were noted in 2 tumors (1\n                    <jats:italic>SFPQ::TFE3\/p53<\/jats:italic>\n                    ; 1\n                    <jats:italic>MTOR\/p53).<\/jats:italic>\n                    \u00a0Metastasis was identified in 5\n                    <jats:italic>TFE3<\/jats:italic>\n                    -rearranged PEComas (OR\u2009=\u20098.7509) and 2\n                    <jats:italic>TSC\/MTOR-<\/jats:italic>\n                    mutated tumors (OR\u2009=\u20090.1143).\n                    <jats:italic>TFE3<\/jats:italic>\n                    -rearranged bladder PEComas show a higher propensity towards aggressive behavior compared to\n                    <jats:italic>TSC\/MTOR-<\/jats:italic>\n                    mutated tumors. Awareness of the molecular signature may be important for prognostic stratification and targeted therapeutic approaches.\n                  <\/jats:p>","DOI":"10.1007\/s00428-025-04250-y","type":"journal-article","created":{"date-parts":[[2025,10,4]],"date-time":"2025-10-04T04:33:10Z","timestamp":1759552390000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Perivascular epithelioid cell tumors of the urinary bladder: a multi-institutional clinicopathologic and molecular analysis of 21 cases"],"prefix":"10.1007","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0874-8964","authenticated-orcid":false,"given":"Ankur R.","family":"Sangoi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anandi","family":"Lobo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ankit","family":"Tiwari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mahmut","family":"Akgul","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shivani","family":"Kandukuri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andres M.","family":"Acosta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Khaleel I.","family":"Al-Obaidy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shilpy","family":"Jha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seema","family":"Kaushal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Swati","family":"Satturwar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jasreman","family":"Dhillon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adeboye O.","family":"Osunkoya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sean R.","family":"Williamson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dinesh","family":"Pradhan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajal B.","family":"Shah","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anil V.","family":"Parwani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liang","family":"Cheng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arndt","family":"Hartmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sambit K.","family":"Mohanty","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abbas","family":"Agaimy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,10,4]]},"reference":[{"issue":"1","key":"4250_CR1","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.humpath.2009.05.011","volume":"41","author":"AL Folpe","year":"2010","unstructured":"Folpe AL, Kwiatkowski DJ (2010) Perivascular epithelioid cell neoplasms: pathology and pathogenesis. 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