{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T01:03:36Z","timestamp":1790643816312,"version":"4.1.0"},"reference-count":36,"publisher":"Springer Science and Business Media LLC","issue":"9","license":[{"start":{"date-parts":[[2026,7,5]],"date-time":"2026-07-05T00:00:00Z","timestamp":1783209600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"},{"start":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T00:00:00Z","timestamp":1790640000000},"content-version":"vor","delay-in-days":86,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Cancer Res Clin Oncol"],"DOI":"10.1007\/s00432-026-06556-z","type":"journal-article","created":{"date-parts":[[2026,7,5]],"date-time":"2026-07-05T13:33:17Z","timestamp":1783258397000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["TFE3-rearranged PEComa with hepatic and pulmonary involvement: diagnostic challenges and resistance to mTOR-targeted therapy"],"prefix":"10.1007","volume":"152","author":[{"given":"Sally","family":"Chahine","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tasnim","family":"Diab","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"El Tannir","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ghassan","family":"Farhat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nawaf","family":"Jurdi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8483-0938","authenticated-orcid":false,"given":"Hazem","family":"I. Assi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,7,5]]},"reference":[{"issue":"4, Supplement","key":"6556_CR7","doi-asserted-by":"publisher","first-page":"A4092","DOI":"10.1016\/j.chest.2024.06.2486","volume":"166","author":"S Acharya","year":"2024","unstructured":"Acharya S et al (2024) Incidental discovery of a perivascular epithelioid cell tumor (PEcoma) in the right lower lung: a case study. Chest 166(4, Supplement):A4092\u2013A4093","journal-title":"Chest"},{"issue":"10","key":"6556_CR3","doi-asserted-by":"publisher","first-page":"1395","DOI":"10.1097\/PAS.0b013e3181f17ac0","volume":"34","author":"P Argani","year":"2010","unstructured":"Argani P et al (2010) A distinctive subset of PEComas harbors TFE3 gene fusions. Am J Surg Pathol vol 34(10):1395\u20131406","journal-title":"Am J Surg Pathol vol"},{"issue":"16","key":"6556_CR29","doi-asserted-by":"publisher","first-page":"2630","DOI":"10.1200\/JCO.2008.18.8391","volume":"27","author":"J Baselga","year":"2009","unstructured":"Baselga J et al (2009) Phase II randomized study of neoadjuvant everolimus plus letrozole compared with placebo plus letrozole in patients with estrogen receptor-positive breast cancer. J Clin Oncol 27(16):2630\u20132637","journal-title":"J Clin Oncol"},{"issue":"6","key":"6556_CR28","doi-asserted-by":"publisher","first-page":"520","DOI":"10.1056\/NEJMoa1109653","volume":"366","author":"J Baselga","year":"2012","unstructured":"Baselga J et al (2012) Everolimus in postmenopausal hormone-receptor-positive advanced breast cancer. N Engl J Med 366(6):520\u2013529","journal-title":"N Engl J Med"},{"issue":"14","key":"6556_CR30","doi-asserted-by":"publisher","first-page":"5319","DOI":"10.1158\/1078-0432.CCR-04-2402","volume":"11","author":"A Boulay","year":"2005","unstructured":"Boulay A et al (2005) Dual inhibition of mTOR and estrogen receptor signaling in vitro induces cell death in models of breast cancer. Clin Cancer Res 11(14):5319\u20135328","journal-title":"Clin Cancer Res"},{"issue":"20","key":"6556_CR21","doi-asserted-by":"publisher","first-page":"5227","DOI":"10.3390\/cancers13205227","volume":"13","author":"A Bourgmayer","year":"2021","unstructured":"Bourgmayer A et al (2021) Natural history and treatment strategies of advanced PEComas: a systematic review. Cancers (Basel) 13(20):5227","journal-title":"Cancers (Basel)"},{"issue":"7","key":"6556_CR11","doi-asserted-by":"publisher","first-page":"746","DOI":"10.1097\/PAT.0b013e32834c768b","volume":"43","author":"AW Chan","year":"2011","unstructured":"Chan AW et al (2011) Primary perivascular epithelioid cell tumour (PEComa) of the urinary bladder. Pathology 43(7):746\u2013749","journal-title":"Pathology"},{"issue":"28","key":"6556_CR19","doi-asserted-by":"publisher","first-page":"e3890","DOI":"10.1097\/MD.0000000000003890","volume":"95","author":"Z Chen","year":"2016","unstructured":"Chen Z et al (2016) A systematic review: perivascular epithelioid cell tumor of gastrointestinal tract. Med (Baltim) 95(28):e3890","journal-title":"Med (Baltim)"},{"issue":"10","key":"6556_CR13","doi-asserted-by":"publisher","first-page":"2249","DOI":"10.1007\/s13555-022-00801-2","volume":"12","author":"PR Cohen","year":"2022","unstructured":"Cohen PR, Kurzrock R (2022) Dermatologic disease-directed targeted therapy (D(3)T(2)): the application of biomarker-based precision medicine for the personalized treatment of skin conditions-precision dermatology. Dermatol Ther (Heidelb) 12(10):2249\u20132271","journal-title":"Dermatol Ther (Heidelb)"},{"key":"6556_CR20","doi-asserted-by":"publisher","first-page":"615","DOI":"10.2147\/CMAR.S351284","volume":"15","author":"AM Czarnecka","year":"2023","unstructured":"Czarnecka AM et al (2023) Management strategies for adults with locally advanced, unresectable or metastatic malignant perivascular epithelioid cell tumor (PEComa): challenges and solutions. Cancer Manag Res 15:615\u2013623","journal-title":"Cancer Manag Res"},{"issue":"12","key":"6556_CR1","doi-asserted-by":"publisher","first-page":"1558","DOI":"10.1097\/01.pas.0000173232.22117.37","volume":"29","author":"AL Folpe","year":"2005","unstructured":"Folpe AL et al (2005) Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol vol 29(12):1558\u20131575","journal-title":"Am J Surg Pathol vol"},{"key":"6556_CR17","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1016\/j.humpath.2023.10.001","volume":"143","author":"M-F Gagnon","year":"2024","unstructured":"Gagnon M-F et al (2024) Molecular alterations in primary and metastatic PEComa: insights from the AACR project GENIE data set. Hum Pathol 143:75\u201376","journal-title":"Hum Pathol"},{"issue":"15\u201316","key":"6556_CR16","doi-asserted-by":"publisher","first-page":"983","DOI":"10.1101\/gad.324657.119","volume":"33","author":"CR Goding","year":"2019","unstructured":"Goding CR, Arnheiter H (2019) MITF-the first 25 years. Genes Dev 33(15\u201316):983\u20131007","journal-title":"Genes Dev"},{"issue":"4, Supplement","key":"6556_CR12","doi-asserted-by":"publisher","first-page":"A4056","DOI":"10.1016\/j.chest.2024.06.2459","volume":"166","author":"S Kaur","year":"2024","unstructured":"Kaur S, Pillai M (2024) Metastatic cardiac PEcoma. Chest 166(4, Supplement):A4056\u2013A4057","journal-title":"Chest"},{"issue":"9","key":"6556_CR14","doi-asserted-by":"publisher","first-page":"1361","DOI":"10.1016\/j.humpath.2007.01.028","volume":"38","author":"H Kenerson","year":"2007","unstructured":"Kenerson H et al (2007) Activation of the mTOR pathway in sporadic angiomyolipomas and other perivascular epithelioid cell neoplasms. Hum Pathol 38(9):1361\u20131371","journal-title":"Hum Pathol"},{"issue":"2","key":"6556_CR6","doi-asserted-by":"publisher","first-page":"315","DOI":"10.1016\/j.path.2022.02.008","volume":"15","author":"EC Kertowidjojo","year":"2022","unstructured":"Kertowidjojo EC, Bennett JA (2022) Update on uterine mesenchymal neoplasms. Surg Pathol Clin 15(2):315\u2013340","journal-title":"Surg Pathol Clin"},{"issue":"5","key":"6556_CR34","first-page":"e14951","volume":"13","author":"P Kopparthy","year":"2021","unstructured":"Kopparthy P, Murphy M (2021) Rapid and durable response with nab-sirolimus after everolimus failure in a patient with perivascular epithelioid cell tumors (PEComas) of the uterus. Cureus 13(5):e14951","journal-title":"Cureus"},{"issue":"1","key":"6556_CR32","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1186\/s40425-018-0415-x","volume":"6","author":"M Lattanzi","year":"2018","unstructured":"Lattanzi M et al (2018) Durable response to anti-PD-1 immunotherapy in epithelioid angiomyolipoma: a report on the successful treatment of a rare malignancy. J Immunother Cancer 6(1):97","journal-title":"J Immunother Cancer"},{"key":"6556_CR18","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1186\/1477-7819-10-213","volume":"10","author":"J Li","year":"2012","unstructured":"Li J, Zhu M, Wang YL (2012) Malignant epithelioid angiomyolipoma of the kidney with pulmonary metastases and p53 gene mutation. World J Surg Oncol 10:213","journal-title":"World J Surg Oncol"},{"issue":"5","key":"6556_CR4","doi-asserted-by":"publisher","first-page":"2453","DOI":"10.1016\/j.asjsur.2024.01.111","volume":"47","author":"J Li","year":"2024","unstructured":"Li J et al (2024) Malignant perivascular epithelioid cell tumor (PEComa) of kidney. Asian J Surg 47(5):2453\u20132454","journal-title":"Asian J Surg"},{"key":"6556_CR25","doi-asserted-by":"publisher","first-page":"641376","DOI":"10.3389\/fonc.2021.641376","volume":"11","author":"A Liapi","year":"2021","unstructured":"Liapi A et al (2021) Inhibitors for uterine metastatic perivascular epithelioid tumors (PEComa) resistant to mTOR inhibitors: a case report and review of literature. Front Oncol 11:641376","journal-title":"Front Oncol"},{"key":"6556_CR9","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1186\/s12886-016-0185-5","volume":"16","author":"CY Lin","year":"2016","unstructured":"Lin CY et al (2016) HMB-45 negative angiomyolipoma of the orbit: a case report and review of the literature. BMC Ophthalmol 16:8","journal-title":"BMC Ophthalmol"},{"issue":"11","key":"6556_CR8","doi-asserted-by":"publisher","first-page":"e250466","DOI":"10.1136\/bcr-2022-250466","volume":"15","author":"BM Marinho","year":"2022","unstructured":"Marinho BM et al (2022) Primary retroperitoneal PEComa: an incidental finding. BMJ Case Rep 15(11):e250466","journal-title":"BMJ Case Rep"},{"issue":"2","key":"6556_CR2","doi-asserted-by":"publisher","first-page":"119","DOI":"10.1007\/s00428-007-0509-1","volume":"452","author":"G Martignoni","year":"2008","unstructured":"Martignoni G et al (2008) PEComas: the past, the present and the future. Virchows Archiv: Int J Pathol vol 452(2):119\u2013132","journal-title":"Virchows Archiv: Int J Pathol vol"},{"issue":"4","key":"6556_CR35","doi-asserted-by":"publisher","first-page":"1001","DOI":"10.3892\/ijo_00000751","volume":"37","author":"Y Nagata","year":"2010","unstructured":"Nagata Y et al (2010) Effect of rapamycin, an mTOR inhibitor, on radiation sensitivity of lung cancer cells having different p53 gene status. Int J Oncol 37(4):1001\u20131010","journal-title":"Int J Oncol"},{"issue":"16","key":"6556_CR22","doi-asserted-by":"publisher","first-page":"3705","DOI":"10.3390\/jcm10163705","volume":"10","author":"T \u015awitaj","year":"2021","unstructured":"\u015awitaj T et al (2021) Efficacy of sirolimus treatment in PEComa-10 years of practice perspective. J Clin Med 10(16):3705","journal-title":"J Clin Med"},{"issue":"17","key":"6556_CR26","doi-asserted-by":"publisher","first-page":"5295","DOI":"10.1158\/1078-0432.CCR-19-0288","volume":"25","author":"R Sanfilippo","year":"2019","unstructured":"Sanfilippo R et al (2019) Role of chemotherapy, VEGFR inhibitors, and mTOR inhibitors in advanced perivascular epithelioid cell tumors (PEComas). Clin Cancer Res 25(17):5295\u20135300","journal-title":"Clin Cancer Res"},{"issue":"20","key":"6556_CR31","doi-asserted-by":"publisher","first-page":"5534","DOI":"10.1158\/1078-0432.CCR-20-1191","volume":"26","author":"R Sanfilippo","year":"2020","unstructured":"Sanfilippo R et al (2020) Addition of antiestrogen treatment in patients with malignant PEComa progressing to mTOR inhibitors. Clin Cancer Res 26(20):5534\u20135538","journal-title":"Clin Cancer Res"},{"issue":"1","key":"6556_CR15","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1002\/cjp2.187","volume":"7","author":"M Schmiester","year":"2021","unstructured":"Schmiester M et al (2021) TFE3 activation in a TSC1-altered malignant PEComa: challenging the dichotomy of the underlying pathogenic mechanisms. J Pathol Clin Res 7(1):3\u20139","journal-title":"J Pathol Clin Res"},{"issue":"10","key":"6556_CR23","doi-asserted-by":"publisher","first-page":"2200","DOI":"10.3390\/jcm10102200","volume":"10","author":"A Sobiborowicz","year":"2021","unstructured":"Sobiborowicz A et al (2021) Feasibility and long-term efficacy of PEComa treatment-20 years of experience. J Clin Med 10(10):2200","journal-title":"J Clin Med"},{"issue":"24","key":"6556_CR24","doi-asserted-by":"publisher","first-page":"e415","DOI":"10.1200\/JCO.2010.29.4678","volume":"28","author":"V Subbiah","year":"2010","unstructured":"Subbiah V, Trent JC, Kurzrock R (2010) Resistance to mammalian target of rapamycin inhibitor therapy in perivascular epithelioid cell tumors. J Clin Oncol 28(24):e415","journal-title":"J Clin Oncol"},{"issue":"2","key":"6556_CR5","doi-asserted-by":"publisher","first-page":"13","DOI":"10.15586\/jkcvhl.v9i2.210","volume":"9","author":"IM Tessone","year":"2022","unstructured":"Tessone IM et al (2022) Massive malignant epithelioid angiomyolipoma of the kidney. J Kidney Cancer VHL 9(2):13\u201318","journal-title":"J Kidney Cancer VHL"},{"issue":"33","key":"6556_CR33","doi-asserted-by":"publisher","first-page":"3660","DOI":"10.1200\/JCO.21.01728","volume":"39","author":"AJ Wagner","year":"2021","unstructured":"Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, Dickson MA (2021) nab-Sirolimus for patients with malignant perivascular epithelioid cell tumors. J Clin Oncol 39(33):3660\u20133670","journal-title":"J Clin Oncol"},{"issue":"5","key":"6556_CR36","doi-asserted-by":"publisher","first-page":"e63","DOI":"10.1016\/j.ijrobp.2021.07.1694","volume":"111","author":"H Willers","year":"2021","unstructured":"Willers H et al (2021) Screening and validation of molecular targeted radiosensitizers. Int J Radiat Oncol Biol Phys 111(5):e63\u2013e74","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"6556_CR27","doi-asserted-by":"publisher","first-page":"582087","DOI":"10.3389\/fonc.2020.582087","volume":"10","author":"J Xu","year":"2020","unstructured":"Xu J et al (2020) Case report: gastrointestinal PEComa With TFE3 rearrangement treated With Anti-VEGFR TKI apatinib. Front Oncol 10:582087","journal-title":"Front Oncol"},{"issue":"22","key":"6556_CR10","doi-asserted-by":"publisher","first-page":"e7050","DOI":"10.1097\/MD.0000000000007050","volume":"96","author":"S Zhang","year":"2017","unstructured":"Zhang S et al (2017) Perivascular epithelial cell tumor (PEComa) of the pancreas: a case report and review of literature. Med (Baltim) 96(22):e7050","journal-title":"Med (Baltim)"}],"container-title":["Journal of Cancer Research and Clinical Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00432-026-06556-z","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00432-026-06556-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00432-026-06556-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T00:27:51Z","timestamp":1790641671000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00432-026-06556-z"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7,5]]},"references-count":36,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2026,9]]}},"alternative-id":["6556"],"URL":"https:\/\/doi.org\/10.1007\/s00432-026-06556-z","relation":{},"ISSN":["1432-1335"],"issn-type":[{"value":"1432-1335","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7,5]]},"assertion":[{"value":"23 March 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"26 June 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"5 July 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no competing interests.","order":1,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The present study complies with the internationally accepted ethical standards. According to local regulations, this study did not require Institutional Review Board approval or consent to participate.","order":2,"name":"Ethics","label":"Ethical approval","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Written informed consent was obtained from the patient for publication of this case report.","order":3,"name":"Ethics","label":"Consent for publication","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"188"}}