{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,24]],"date-time":"2026-02-24T08:28:58Z","timestamp":1771921738158,"version":"3.50.1"},"reference-count":42,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T00:00:00Z","timestamp":1768435200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Pulmonary blastoma (PB) is an exceedingly rare and aggressive primary lung malignancy. Classic biphasic pulmonary blastoma (CBPB), a subtype, is associated with a historically poor prognosis, particularly in advanced stages. There is no standard treatment for advanced CBPB, and reports of long-term survival are exceptionally rare. We present a case of prolonged disease-free survival in an adult with stage IIIC CBPB and review contemporary long-term survivors to identify prognostic factors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>A 32-year-old female presented with cough and dyspnea. Imaging revealed a large mass in the left upper lobe with mediastinal and supraclavicular lymph node involvement, leading to a diagnosis of stage IIIC CBPB. The patient received six cycles of neoadjuvant chemotherapy with ifosfamide plus epirubicin (IFO + EPI) and achieved a partial response. She subsequently underwent left upper lobectomy and lymph node dissection. Due to dense adhesion of mediastinal lymph node station 5 to the aortic arch, phrenic nerve, and vagus nerve, complete resection was not feasible, resulting in R2 resection (macroscopic residual disease). Postoperatively, she completed adjuvant chemoradiotherapy (one cycle of IFO + EPI, concurrent radiotherapy 54 Gy combined with docetaxel plus carboplatin, and two cycles of docetaxel plus cisplatin). As of July 2025, the patient has remained disease-free for more than six years post-diagnosis, demonstrating exceptional long-term survival.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights that aggressive multimodal therapy, including neoadjuvant chemotherapy, surgical resection, and adjuvant radiotherapy, can lead to long-term survival in advanced CBPB patients. A literature review (from 2000 to present) revealed 13 CBPB patients surviving &amp;gt; 3 years, with 5 of them surviving \u2265 5 years. Most of these patients underwent surgery and multimodal treatment. Aggressive local therapy for recurrent disease and antiangiogenic agents and immunotherapy represent promising strategies to improve outcomes in this rare malignancy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fonc.2025.1727839","type":"journal-article","created":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T05:12:38Z","timestamp":1768453958000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Exceptional long-term survival in an adult with advanced classic biphasic pulmonary blastoma: a case report and literature review"],"prefix":"10.3389","volume":"15","author":[{"given":"Jun","family":"Li","sequence":"first","affiliation":[{"name":"Department of Oncology, Wuhan Pulmonary Hospital","place":["Wuhan, Hubei, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Si","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Oncology, Wuhan Pulmonary Hospital","place":["Wuhan, Hubei, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qiliang","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Pathology, Wuhan Pulmonary Hospital","place":["Wuhan, Hubei, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ming","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Oncology, Wuhan Pulmonary Hospital","place":["Wuhan, Hubei, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,1,15]]},"reference":[{"key":"B1","article-title":"Pulmonary blastoma","volume":"17","author":"Hussain","year":"2007","journal-title":"J Coll Physicians Surg\u2013Pak JCPSP"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1111\/crj.13701","article-title":"Classic biphasic pulmonary blastoma: A case report and review of the literature from 2000 to 2022","volume":"17","author":"Yao","year":"2023","journal-title":"Clin Respir J"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1016\/j.lungcan.2011.03.018","article-title":"Classic biphasic pulmonary blastoma: a case report and review of the literature","volume":"73","author":"Van Loo","year":"2011","journal-title":"Lung Cancer Amst Neth"},{"key":"B4","doi-asserted-by":"publisher","first-page":"20963","DOI":"10.1038\/s41598-025-08571-5","article-title":"Epidemiological characteristics and prognostic risk factors of pulmonary blastoma based on the 2021 WHO classification","volume":"15","author":"Yang","year":"2025","journal-title":"Sci Rep"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4615-2630-8_16","article-title":"Pulmonary blastomas","volume":"72","author":"Koss","year":"1995","journal-title":"Cancer Treat Res"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1016\/s0003-4975(02)03494-x","article-title":"Pulmonary blastoma: medium-term results from a regional center","volume":"73","author":"Zaidi","year":"2002","journal-title":"Ann Thorac Surg"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtho.2021.11.003","article-title":"The 2021 WHO classification of lung tumors: impact of advances since 2015","volume":"17","author":"Nicholson","year":"2022","journal-title":"J Thorac Oncol Off Publ Int Assoc Study Lung Cancer"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.18999\/nagjms.78.4.507","article-title":"What do we know about pulmonary blastoma?: review of literature and clinical case report","volume":"78","author":"Brodowska-Kania","year":"2016","journal-title":"Nagoya J Med Sci"},{"key":"B9","article-title":"Pulmonary blastoma. 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