{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T08:05:52Z","timestamp":1753862752582,"version":"3.41.2"},"reference-count":49,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,12,22]],"date-time":"2023-12-22T00:00:00Z","timestamp":1703203200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Medicine"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>The advances in immune checkpoint inhibitors (ICIs) were relatively slow in rare tumors. Therefore, we conducted a multi\u2010center study evaluating the efficacy of ICI monotherapy and the combination of ICIs with chemotherapy (CT) in patients with advanced rare tumors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this retrospective cohort study, we included 93 patients treated with ICIs for NCI\u2010defined rare tumors from the 12 cancer centers in Turkey. The primary endpoints were the overall response (ORR) and disease control rate (DCR).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The cohort's median age was 56, and 53.8% of the patients were male. The most frequent diagnosis was sarcoma (29%), and 81.7% of the patients were previously treated with at least one line of systemic therapy in the advanced stage.<\/jats:p><jats:p>The ORR and DCR were 36.8% and 63.2%, respectively. The germ cell tumors had the lowest ORR (0%), while the Merkel cell carcinoma had the highest ORR to ICIs (57.1%). Patients treated with ICI\u2009+\u2009ICI or ICI plus chemotherapy combinations had higher ORR (55.2% vs. 27.6%, <jats:italic>p<\/jats:italic>\u2009=\u20090.012) and DCR (82.8% vs. 53.4%, <jats:italic>p<\/jats:italic>\u2009=\u20090.008).<\/jats:p><jats:p>The median OS was 13.47 (95% CI: 7.79\u201319.15) months, and the six and 12\u2010month survival rates were 71% and 52%. The median duration of response was 16.59\u2009months, and the 12\u2010month progression\u2010free survival rate was 66% in responders. The median time\u2010to\u2010treatment failure was 5.06\u2009months (95% CI: 3.42\u20136.71). Three patients had high\u2010grade irAEs with ICIs (grade 3 colitis, grade 3 gastritis, and grade 3 encephalitis in one patient each).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We observed over 30% ORR and a 13\u2010month median OS in patients with rare cancers treated with ICI monotherapy or ICI plus CT combinations. The response rates to ICIs or ICIs plus CT significantly varied across different tumor types. Responding patients had over 2\u2009years of survival, highlighting a need for further trials with ICIs for patients with rare tumors.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cam4.6869","type":"journal-article","created":{"date-parts":[[2023,12,23]],"date-time":"2023-12-23T06:40:43Z","timestamp":1703313643000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The efficacy of immunotherapy and chemoimmunotherapy in patients with advanced rare tumors: A Turkish oncology group (<scp>TOG<\/scp>) study"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6924-9467","authenticated-orcid":false,"given":"Deniz Can","family":"Guven","sequence":"first","affiliation":[{"name":"Department of Medical Oncology Hacettepe University Cancer Institute  Ankara Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Musa 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