Type III pleuropulmonary blastoma in a toddler: a case report

Pleuropulmonary blastoma (PPB) is an exceptionally rare and aggressive pediatric thoracic malignancy, accounting for less than 1 % of childhood lung tumors. Its nonspecific respiratory presentation often mimics common conditions, leading to delayed recognition, particularly in resource-limited settings. A 2.5-year-old female with progressive respiratory distress since infancy managed as recurrent pneumonias without improvement was admitted to our hospital. She was tachypneic with absent air entry over the right hemithorax. Laboratory studies revealed leukocytosis and anemia. A contrast-enhanced chest computerized tomography (CT) showed a large heterogeneously enhancing right lower lobe mass with necrotic foci, encasement of hilar broncho-vascular structures, associated pleural and pericardial effusions, and mediastinal shift suspicious for a malignant process. Brain magnetic resonance (MRI) and abdominal CT were done to assess for metastasis and were normal. Given the child's critical condition, upfront surgical resection was undertaken without preoperative biopsy or neoadjuvant therapy. We did a right thoracotomy and found a large, encapsulated mass arising from the right lower lobe, which was excised via a lobectomy. Histopathology confirmed a type III pleuropulmonary blastoma, demonstrating sheets of undifferentiated mesenchymal cells with pleomorphism and necrosis. The patient had an uneventful postoperative recovery and was subsequently referred to a specialized oncology center for adjuvant chemotherapy. Pleuropulmonary blastoma should be considered in the differential diagnosis of toddlers who present with recurrent or persistent respiratory symptoms that fail to improve with standard therapies.