Pleuropulmonary blastoma: four cases

Pleuropulmonary blastoma (PPB) is a rare malignant neoplasm affecting child ren. A retrospective review was carried out of patients diagnosed as having PPB at one institute over a period of 16 years. The presentation, diagnosi s, treatment, and family history were studied. PPB usually presents with sy mptoms and signs of respiratory tract infection, and the diagnosis may be d elayed. There are no distinguishing clinical features or imaging studies. T he diagnosis is made on histologic examination of tumour material and is so metimes difficult to differentiate from benign cystic lung lesions. The tre atment is primarily complete excision of the tumour, followed in some cases by intense chemotherapy. PPB is a strong predictor of the presence of tumo urs in close relatives. Four patients treated at our institute are discusse d along with a review of the literature.