Pleuropulmonary blastoma: Cystic tumour misdiagnosed as an emphysematous bulla

Abstract Pleuropulmonary blastoma is a dysontogenetic neoplasm of childhood, histologically characterized by a primitive variably mixed blastematous and sarcomatous appearance. It presents a pattern of rapid growth and is associated with a poor prognosis. We report the case of a 13-month-old girl, consulting for wheezing, dyspnea and loss of appetite. Chest X-ray revealed a mediastinal deviation with a clear appearance of the right lung. Computed tomography showed a voluminous emphysematous bulla of the right lung and atelectasis of the lower lobe. The final diagnosis was made on the basis of histological features of a cystic tumour, showing blastematous elements associated with sarcomatous compound confirming the diagnosis of pleuropulmonary blastoma type I. Subsequent chemotherapy was performed. Four years after the operation, the child is well with no evidence of disease recurrence or metastasis.