Bashiri Aliabadi Somayeh, Baghersalimi Adel, Darbandi Bahram, Fallah Arzpeyma Sima, Enshaei Mercedeh
Burn and Regenerative Medicine Research Center Guilan University of Medical Sciences Rasht Iran.
Pediatric Diseases Research Center Guilan University of Medical Sciences Rasht Iran.
Clin Case Rep. 2025 Sep 10;13(9):e70843. doi: 10.1002/ccr3.70843. eCollection 2025 Sep.
Pleuropulmonary blastoma (PPB) is a rare, highly aggressive pulmonary tumor that typically presents in the pediatric population. The overall prognosis of PPB is poor, although type I PPB can regress spontaneously. In this case report, the authors present a 3.5-year-old boy histopathologically diagnosed with PPB type III, who underwent three courses of the ifosfamide, vincristine, actinomycin D, and doxorubicin (IVADO) chemotherapy regimen after surgical resection. Although the initial post-chemotherapy evaluations showed no residual or metastatic tumor, based on PET-CT scan findings, it relapsed 4 months after the last chemotherapy course. Finally, the patient expired due to disease progression. In addition to the case presentation, the authors provide an overview of PPB and discuss how this case illustrates key diagnostic and management challenges in advanced PPB type III.
胸膜肺母细胞瘤（PPB）是一种罕见的、侵袭性很强的肺部肿瘤，通常发生于儿童群体。PPB的总体预后较差，不过I型PPB可自发消退。在本病例报告中，作者介绍了一名3.5岁组织病理学诊断为III型PPB的男孩，该男孩在手术切除后接受了三个疗程的异环磷酰胺、长春新碱、放线菌素D和阿霉素（IVADO）化疗方案。尽管化疗后的初始评估显示没有残留或转移性肿瘤，但根据PET-CT扫描结果，在最后一个化疗疗程后4个月复发。最终，患者因疾病进展而死亡。除了病例介绍外，作者还对PPB进行了概述，并讨论了该病例如何说明了晚期III型PPB的关键诊断和管理挑战。
