Solid (Type 3) pleuropulmonary blastoma

Abstract

Introduction: Pleuropulmonary blastoma is a rare solid and/or cystic progressive and aggressive malignant intra-thoracic tumor of childhood which belongs to a group of dysontogenetic neoplasms such as Wilms tumor, neuroblastoma and hepatoblastoma. The incidence of pleuropulmonary blastoma ranges between 0.25 and 0.5% of primary lung tumors making this entity a rare primary malignant lung tumor. Case Report: A two-month-old male neonate was referred from a regional hospital with a three-day history of cough, fever and shortness of breath. No previous medical history reported. The patient was delivered at term through a normal vaginal delivery which was uneventful. Birth weight 3 kg, length 48 cm, Apgar score 10/10 and the head circumference was 35 cm. The patient's retro-viral disease status was negative. On percussion there was dullness and bilateral crepitations. There was bilateral decreased air-entry. No signs of cardiac failure. The rest of the systems were within normal limits. The arterial blood gases showed respiratory acidosis with hypoxia. Chest X-ray showed a white out of the right hemithorax and CT scan of the chest showed a large soft tissue mass in the right hemithorax associated with free fluid in the right hemithorax and the biopsy was performed which confirmed the diagnosis of a pleuropulmonary blastoma. The pleuropulmonary blastoma was a solid type (Type 3). Conclusion: Pleuropulmonary blastoma poses a diagnostic challenge because it does not have specific clinical or radiological features. The late diagnosis and delayed treatment of pleuropulmonary blastoma are associated with a deadly prognosis.