Exceptional long-term survival in an adult with advanced classic biphasic pulmonary blastoma: a case report and literature review
Year: 2026
Type: article
Study design: Case Report
Abstract: Background: 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. Case presentation: 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. Conclusion: 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 > 3 years, with 5 of them surviving ≥ 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.
Source: Frontiers in Oncology
Authors: Jun Li, Si Chen, Qiliang Liu, Ming Li
Institution: Wuhan Pulmonary Hospital
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,801
OpenAlex ID: W7124319204