Clinical Characteristics, Diagnosis, and Management of Primary Malignant Lung Tumors in Children: A Single-Center Analysis

Background/Objectives: This study aims to present a diagnostic and therapeutic approach to pediatric primary lung tumors based on a single-center experience, highlighting the role of postoperative histopathological and genetic testing in identifying novel chromosomal rearrangements and supporting the application of targeted therapies. Methods: We retrospectively reviewed medical records of children with primary pulmonary tumors treated from 2015–2025. Diagnostic evaluation included laboratory tests, chest imaging, bronchoscopy with biopsy, and histopathological/immunohistochemical analyses. Treatment encompassed surgical resection and, when needed, chemo-, radio-, or targeted therapies based on tumor characteristics. Results: Seven pediatric patients (aged 2–18 years) were included, with inflammatory myofibroblastic tumor (IMT) being the most common diagnosis (3/7 cases, 43%). Tumor types were heterogeneously distributed. The majority of patients presented with nonspecific symptoms, predominantly dry cough (86%). Chest CT scans were essential for tumor localization and staging, while bronchoscopy contributed significantly to both diagnosis and initial management in cases with intraluminal involvement. Radical surgical resection was the predominant treatment, especially for pleuropulmonary blastoma (PPB) and IMT. In selected cases, neoadjuvant therapy targeting documented fusion gene rearrangements facilitated tumor shrinkage prior to surgery. The two-year survival rate was 66.7%, with four survivors, two deaths due to disseminated disease, and one patient lost to follow-up. Conclusions: A multidisciplinary diagnostic approach integrating radiologic, bronchoscopic, histopathological, and genetic testing ensures high diagnostic accuracy. While conventional treatments remain curative in many cases, targeted therapies directed at key molecular alterations may offer critical therapeutic options for selected patients.