Pleuropulmonary blastoma in A 4-year-Old male: A case report

Abstract

Introduction

Pleuropulmonary blastoma (PPB) in children can present with symptoms such as chest or abdominal pain, cough, fever, and respiratory distress, often leading to misdiagnosis as infections, pneumothorax, or pneumonia.

Case presentation

A 4-year-old male presented with generalized abdominal pain, hematuria, and respiratory symptoms. chest pain, cough, fever, and chills. Despite outpatient treatment with antibiotics, his condition failed to improve, and he was admitted to the hospital. Chest X-ray showed that the right hemithorax was whited out. Chest ultrasound a mass-like area in the right lower lobe. A chest computed tomography (CT) showed a large pleural effusion and a completely collapsed right lung. Laboratory tests showed elevated inflammatory markers and leukocytosis. Contrast-enhanced computed tomography (CECT) showed a large, well-defined heterogeneous mass measuring 98 × 86 mm in the right hemithorax. He was taken to the operating room for a resection. Through a right thoracotomy we found and resected a tumor measuring 3 × 14 × 16 cm arising from the lower lobe, and we resected a small portion of a rib that appeared involved by the tumor. Postoperative studies showed metastatic disease in several ribs. Pleural fluid cytology showed malignant cells, and the histopathology of the specimen confirmed the diagnosis of a type III PPB. Genetic testing was positive for a DICER1 mutation. Chemotherapy with ifosfamide, vincristine, actinomycin-D and doxorubicin was initiated five days after diagnosis. He currently continues his treatment.

Conclusion

Chest tumors should be suspected in patients that present respiratory symptoms that do not respond to standard measures and have abnormal findings on plain chest films. PPB should be suspected if a lung mass is found. Patients with confirmed PPB must undergo genetic testing to rule out DICER1 mutations, which are associated with a variety of other malignancies.