Background and aims: Primary lung tumors (PLTs) in children are rare, and surgery remains the key to ensure remission. Here we describe the PLTs clinical characteristics, their management, and the pulmonary outcome following surgery.
Methods: We carried out a French national cohort of pediatric PLTs from 2013 to 2023 from the FRACTURE rare pediatric tumors national database. We included children under 18 years at diagnosis who underwent surgery for a histologically proven PLT, with a minimum of 6 months of follow-up (FU) post surgery.
Results: Sixty-two patients were included. The median age at diagnosis was 3.6 years [3; 11], sex ratio 1.07. Pleuropulmonary blastoma was the most frequent tumor retrieved (n = 31). Sixty patients underwent surgery: 32 lobectomies, 15 wedges, five segmentectomies, and five pneumectomies. A thoracoscopic approach was carried out in 14% of the cases. At 6 months post surgery and at the last follow-up (median time of 5.7 years [3.4; 7.6]), respectively, 11 and eight patients presented with pulmonary symptoms, and 10 and three patients presented with surgical complications. During the post-surgery period, 22 children benefited from an evaluation of their respiratory function by pulmonary function tests, and four of them remained with abnormal results.
Conclusions: Surgery is key to ensure remission in PLTs and seems secure. However, respiratory symptoms are noted in 13% of children during the FU, and this rate is probably underestimated. Therefore, we suggest a systematic pulmonary FU to optimize postoperative pulmonary rehabilitation and, therefore, the child's pulmonary outcome.
Keywords: lung cancer in children; primary lung tumor in children; pulmonary follow‐up in children with lung tumor; surgical approach in children's lung tumor.
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