{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T12:21:41Z","timestamp":1788351701501,"version":"build-2803163510"},"reference-count":22,"publisher":"BMJ","issue":"7","license":[{"start":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T00:00:00Z","timestamp":1785283200000},"content-version":"unspecified","delay-in-days":28,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000090","name":"Congressionally Directed Medical Research Programs","doi-asserted-by":"publisher","award":["W81XWH-21-1-0942"],"award-info":[{"award-number":["W81XWH-21-1-0942"]}],"id":[{"id":"10.13039\/100000090","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100003064","name":"Kidney Cancer Association","doi-asserted-by":"publisher","award":["KCA-2025-PED-001"],"award-info":[{"award-number":["KCA-2025-PED-001"]}],"id":[{"id":"10.13039\/100003064","id-type":"DOI","asserted-by":"publisher"}]},{"name":"NIH","award":["P30-CA008748"],"award-info":[{"award-number":["P30-CA008748"]}]},{"name":"NCI","award":["5K08CA272960-03"],"award-info":[{"award-number":["5K08CA272960-03"]}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Immunother Cancer"],"accepted":{"date-parts":[[2026,7,17]]},"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:p>\n                    Epithelioid angiomyolipoma (E-AML) is a rare renal tumor, which can exhibit malignant potential. Because angiomyolipomas frequently harbor\n                    <jats:italic>TSC1\/TSC2<\/jats:italic>\n                    alterations, mTOR inhibitors are commonly used. However, durable disease control remains inconsistent. The potential for long-lasting response with immune checkpoint inhibitors supports exploring their role in E-AML. We report a patient with highly symptomatic, locally advanced renal E-AML treated with first-line ipilimumab plus nivolumab. The patient experienced rapid symptomatic and radiographic improvement after treatment initiation. She subsequently developed immune-related sarcoidosis, which resolved after discontinuation of immunotherapy and with corticosteroids. Given sustained disease control, she underwent delayed nephrectomy demonstrating complete pathologic response in the primary tumor. Genomic analysis revealed a germline mutation in\n                    <jats:italic>FLCN<\/jats:italic>\n                    consistent with Birt-Hogg-Dub\u00e9 syndrome. T-cell receptor sequencing identified a shared expanded T-cell clone in peripheral blood and within the tumor. With extended follow-up for over 5 years, she remains in durable remission after dual immune checkpoint blockade. To our knowledge, this is the first report of dual immune checkpoint blockade in renal E-AML. This case demonstrates that first-line ipilimumab plus nivolumab can induce deep, durable response\u2014including complete pathologic response\u2014in a rare malignancy with limited standard treatment options, supporting further evaluation of immunotherapy in this disease.\n                  <\/jats:p>","DOI":"10.1136\/jitc-2026-015794","type":"journal-article","created":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T11:10:27Z","timestamp":1785323427000},"page":"e015794","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Complete response to dual immune checkpoint blockade in a patient with Birt-Hogg-Dub\u00e9 syndrome and epithelioid angiomyolipoma"],"prefix":"10.1136","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-1673-5389","authenticated-orcid":false,"given":"Juliana","family":"Viola","sequence":"first","affiliation":[{"name":"SUNY Downstate Health Sciences University, Brooklyn, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Cornish","sequence":"additional","affiliation":[{"name":"Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria I","family":"Carlo","sequence":"additional","affiliation":[{"name":"Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anis","family":"Hamid","sequence":"additional","affiliation":[{"name":"Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor E","family":"Reuter","sequence":"additional","affiliation":[{"name":"Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Russo","sequence":"additional","affiliation":[{"name":"Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6925-2327","authenticated-orcid":false,"given":"Robert J","family":"Motzer","sequence":"additional","affiliation":[{"name":"Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0551-5807","authenticated-orcid":false,"given":"Martin H","family":"Voss","sequence":"additional","affiliation":[{"name":"Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0223-3479","authenticated-orcid":false,"given":"Ritesh R","family":"Kotecha","sequence":"additional","affiliation":[{"name":"Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,7,29]]},"reference":[{"key":"2026090205001992000_14.7.e015794.1","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","year":"2010","journal-title":"Am J Surg 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