| Summary: | Purpose: Use of extrapleural pneumonectomy for malignancy in children is rarely performed with few descriptions in the literature. In this article, we describe a 19-month-old girl who underwent an extrapleural pneumonectomy with enbloc resection of the diaphragm and pericardium for advanced pleuropulmonary blastoma. We achieved an R0 resection and survival without local disease recurrence at 23 months. Conclusion: Extrapleural pneumonectomy is feasible in some circumstances to achieve oncologic control in cases of advanced PPB. This should be kept as part of the surgeons' armamentarium. Keywords: Pleuropulmonary blastoma, Tension physiology, Pneumonectomy, Extrapleural pneumonectomy
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