A 4-year-old girl presented with an intrathoracic tumour that was pleural in origin, invading the right lower lobe. The patient underwent urgent right thoracotomy and surgical excision of the tumour and right lower lobectomy as the tumour was invading that lobe. The histological report initially reported it as a rhabdomyosarcoma, embryonal type, but after consultation it was changed to type III pleuropulmonary blastoma. The patient was treated with chemotherapy but succumbed to the disease 12 months after the diagnosis. Relevant literature on pleuropulmonary blastoma is reviewed.
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