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Pleuropulmonary blastoma (PPB) is a rare malignant mesenchymal tumor. It is classified into 3 subgroups, and PPB type 1 is known to be a cystic lesion with good prognosis. Here, we report a case of PPB type 1 seen in a 1-year-old girl whose pulmonary cystic lesion had been followed-up as a congenital pulmonary airway malformation since neonate. The cyst had been diagnosed as congenital pulmonary airway malformation before surgery but the final diagnosis was type 1 PPB. The tumor had a somatic mutation in exon 25 of the DICER 1 gene whereas no germline mutation was found.

Purposes: Intralobar pulmonary sequestration is a rare congenital anomaly that causes nonspecific respiratory symptoms. This report presented our surgical experience for pediatric cases of intralobar pulmonary sequestration in our institute. Methods: Between 2002 and 2014, 30 pediatric patients with intralobar pulmonary sequestration underwent lobectomy. The clinical characteristics of patients were retrospectively analyzed using medical records and imaging data. Results: The median age at operation was 23 months. The sequestered lung was situated in the right lower lung in 15 cases and in the left lower lung in 15 cases. Eight cases were prenatally diagnosed by fetal ultrasound and magnetic resonance imaging. One aberrant artery was seen in 26 cases and two aberrant arteries in 4 cases. The drainage vein from the sequestered lung was: 1) the pulmonary vein in 27 cases; 2) the inferior vena cava in 1 case; and 3) the azygos vein and the pulmonary vein in 2 cases. All patients underwent lower lobectomy of the affected side without postoperative early and long-term complications. Conclusion: Due to the increased rate of prenatal diagnoses, the age at operation has decreased. With respect to safety, it is vital to ascertain the number of aberrant arteries and the venous drainage route before lobectomy.