Pleuropulmonary blastoma in a 3-year-old girl: A report of a rare case and review of the literature

Pleuropulmonary blastoma (PPB) is a rare, pediatric soft-tissue sarcoma that mainly occurs in the pleural cavity or lungs. Patients with PPB present with nonspecific symptoms mimicking pneumonia or respiratory distress syndrome. Herein, we report a 3-year-old, female case of PPB presenting with fever, cough, chest pain, and progressive shortness of breath. Chest X-ray demonstrated near complete opacification of the right hemithorax with a mild shift of mediastinum to the left, which could be due to large, right-sided pleural effusion. However, any underlying pneumonic consolidation or mass could not be excluded. On ultrasound, small right-sided pleural effusion was confirmed. However, complex cystic mass with fine septation within the pleural cavity was also identified. A recommendation was made to place a chest tube to decrease the compressive effect on the cardiovascular system and contralateral lung and help the patient with his worsening respiratory compromise. Two days after, initial insertion of the chest tube, tissue fragments were draining out from the tube, which was sent for pathologic evaluation. Histopathologic examination revealed a tumoral lesion composed of spindle cells admixed with islands of cartilage and primitive dark cells, which showed a positive reaction to S-100, vimentin, and desmin in immunohistochemical staining. The findings were consistent with a diagnosis of PPB. The patient received neoadjuvant therapy and then underwent surgical resection. The main interesting feature of the case was tissue collection from the chest tube with no need for biopsy or any other invasive procedure to obtain the tissue samples required for histopathologic analysis.