Hypertensive pneumothorax with cystic lesions: Pleuropulmonary blastoma in an infant

Pleuropulmonary blastoma (PPB) is a rare primitive malignant lung cancer that occurs in pedi-atric age. Its main differential diagnosis is congenital cystic pulmonary malformation (CPAM).A 30-day-old infant with respiratory failure obtained a chest x-ray and a computed tomogra-phy scan (CT) which revealed hypertensive pneumothorax with multifocal bilateral cysts. After thoracic drainage, the patient underwent multiple thoracoscopic pulmonary resections. The first histological diagnosis was of type 2 CPAM.During the radiological follow-up, an increase in the number and dimension of the lesions was detected. Thus, a histological revision was performed, leading to the diagnosis of type I PPB, at nine months. The patient subsequently underwent chemotherapy.At the five-year follow-up appointment, chest magnetic resonance (MR) and CT scans showed a dimensional increase in size of the lesions, with the risk of recurrent pneumothorax. An upper right lobectomy and wedge resection of the residual cysts were performed.Control MR scans showed normalization of the lung parenchyma and the patient showed sub-stantial clinical improvement.

Automated summary

A 30-day-old infant with respiratory failure was diagnosed with hypertensive pneumothorax and congenital cystic pulmonary malformation (CPAM). After surgery, the initial diagnosis was revised to pleuropulmonary blastoma (PPB). The patient underwent chemotherapy followed by lobectomy and wedge resection, resulting in clinical improvement and normalization of lung parenchyma on imaging.