Pleuropulmonary blastoma (type III) in a two-year-old: A cas
Pleuropulmonary blastoma is a rare, aggressive, malignant tumor of the lungs or pleura that primarily affects children. Pleuropulmonary blastoma is classified into 3 types based on morphology, including cystic (type I), mixed (type II), or solid (type III). These morphological types correlate with prognosis.

A 2-year-old girl presented to the hospital with increasing breathlessness for 1 month. On admission, she had a temperature of 37°C, a respiratory rate of 60 breaths/min, and oxygen saturation of 88% on room air. No focal neurological deficit was noted. Breath sounds were absent in the right lung. Radiography demonstrated the complete opacification of the right hemithorax, the trachea, and the left and right main bronchi, and the mediastinum was shifted to the left. A chest computed tomography (CT) scan revealed a large, heterogeneous mass (measuring 10 × 12 × 14 cm), pushing the heart to the left. No coastal erosion was identified. Spine and brain magnetic resonance imaging (MRI) appeared normal. The patient underwent a tumor biopsy, and the histopathology result suggested a PPB. The patient underwent tumoral resection, and the histological result confirmed a solid PPB. The patient received adjuvant chemotherapy with vincristine, ifosfamide, etoposide, actinomycin-D, and cyclophosphamide. However, 5 months later, the patient appeared paraplegic. Chest X-ray showed opacity on the right lung, and CT scans revealed a heterogenous enhancing mass in the periphery of the right lung. Spinal MRI showed multiple vertebral body lesions, which extended into the spinal canal.

Breathlessness is the most common symptom of PPB, and others include pulmonary infection, cough, and abdominal pain. Pneumothorax is present in up to 30% of type I cases. Imaging features are variable, depending on the tumor type. Type I PPB presents as multiloculated, air-filled lung cysts with thin septa often located in the periphery of the lung. However, type I PPB can be difficult to differentiate from congenital pulmonary airway malformations. Type II PPB presents as a solid-cystic tumor, and type III PPB presents as a solid tumor that may invade the adjacent organs.

Source:https://www.sciencedirect.com/science/article/pii/S1930043321005069?dgcid=rss_sd_all
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