Primary bronchopulmonary leiomyosarcoma in childhood

There have been only nine cases of primary bronchopulmonary leiomyosarcoma (PBPL) in children reported in the English language literature, to our knowledge. Herein, we report the tenth case of PBPL in a 24-hour-old newborn infant with associated nonimmunologic hydrops fetalis. We believe this to be the youngest patient ever reported to have PBPL, and the first such tumor, to our knowledge, to be a possible cause of nonimmunologic hydrops fetalis. The patient was alive and well 34 months after a right-sided lower lobectomy, without the need for radiotherapy and/or chemotherapy. We review the clinical pathologic features, treatment, and follow-up of PBPL.