Borgia Paola, Cafferata Barbara, Paratore Claudio, Anfigeno Lorenzo, Conte Alessio, Florio Angelo, Gallizia Annalisa, Del Monte Marco, Buffelli Francesca, Rizzo Francesca, Damasio Maria Beatrice, Salvati Pietro, Perri Katia, Garaventa Alberto, Battaglia Teresa, Livellara Virginia, Conte Massimo, Rossi Giovanni Arturo, Vellone Valerio Gaetano, Torre Michele, Castellani Carlo, Sacco Oliviero
Pediatric Pulmonology and Respiratory Endoscopy Unit, IRCCS Istituto Giannina Gaslini, 16147 Genova, Italy.
Pathology Unit, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy.
J Clin Med. 2025 Mar 22;14(7):2173. doi: 10.3390/jcm14072173.
: Primary lung tumors in pediatric patients are rare, predominantly malignant, and present diagnostic challenges due to symptom overlap with more common conditions such as inflammatory processes or asthma. Evidence-based approaches for managing these rare neoplasms in childhood are scarce. This retrospective study reports the experience of a pediatric referral center in diagnosing and treating these tumors. Pediatric primary lung tumors treated at Giannina Gaslini Children's Hospital between January 2016 and January 2024 were included. Data on clinical presentation, histopathology, imaging, treatment approaches, and outcomes were systematically collected and analyzed. Nine patients (six males and three females) were identified, with a mean age (±SD) at diagnosis of 8.81 ± 5 years. The most common clinical manifestation was recurrent pneumonia (four patients), followed by persistent cough and wheezing (three patients). The average duration of symptoms before diagnosis was 12.8 months ± 12.2 months. Histopathological diagnoses were typical carcinoid tumors ( = 2), atypical carcinoid tumors ( = 2), inflammatory myofibroblastic tumors ( = 2), congenital peribronchial myofibroblastic tumor ( = 1), myoepithelial carcinoma ( = 1), and pleuropulmonary blastoma ( = 1). Radical surgery resulted in complete response for seven patients, with a median follow-up of 52 months (IQR 39 months). The myoepithelial carcinoma was treated with multimodal therapy, relapsed after 17 months, and adjuvant chemotherapy is currently ongoing. Neoadjuvant chemotherapy for the pleuropulmonary blastoma is currently ongoing. Primary lung tumors in children, though rare, may have favorable outcomes when appropriately managed. Nonspecific clinical presentations often contribute to diagnostic delays. This study highlights the critical need of thorough evaluation in cases of persistent, therapy-resistant aspecific respiratory symptoms. Early diagnosis, coupled with complete surgical resection, significantly improves prognosis.
小儿原发性肺肿瘤较为罕见，多为恶性，且因与炎症或哮喘等更常见病症症状重叠而带来诊断挑战。针对儿童期这些罕见肿瘤的循证管理方法匮乏。这项回顾性研究报告了一家儿科转诊中心诊断和治疗这些肿瘤的经验。纳入了2016年1月至2024年1月期间在吉安尼娜·加斯利尼儿童医院接受治疗的小儿原发性肺肿瘤患者。系统收集并分析了临床表现、组织病理学、影像学、治疗方法及预后等数据。共确定了9例患者（6例男性和3例女性），诊断时的平均年龄（±标准差）为8.81±5岁。最常见的临床表现是反复肺炎（4例患者），其次是持续性咳嗽和喘息（3例患者）。诊断前症状的平均持续时间为12.8个月±12.2个月。组织病理学诊断为典型类癌肿瘤（=2）、非典型类癌肿瘤（=2）、炎性肌纤维母细胞瘤（=2）、先天性支气管周围肌纤维母细胞瘤（=1）、肌上皮癌（=1）和胸膜肺母细胞瘤（=1）。根治性手术使7例患者完全缓解，中位随访时间为52个月（四分位间距39个月）。肌上皮癌采用多模式治疗，17个月后复发，目前正在进行辅助化疗。胸膜肺母细胞瘤的新辅助化疗目前正在进行。儿童原发性肺肿瘤虽罕见，但经适当管理可能有良好预后。非特异性临床表现常导致诊断延迟。本研究强调了对持续性、治疗抵抗性非特异性呼吸道症状病例进行全面评估的迫切需求。早期诊断并结合完整的手术切除可显著改善预后。
