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Primary bronchopulmonary leiomyosarcoma in childhood
Insights
This study reports the tenth case of pediatric primary bronchopulmonary leiomyosarcoma (PBPL) in a newborn, potentially causing nonimmunologic hydrops fetalis. The infant remained healthy 34 months post-surgery without further treatment.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Rare Tumors
Background:
- Primary bronchopulmonary leiomyosarcoma (PBPL) is an extremely rare pediatric malignancy.
- Fewer than ten cases have been documented in English literature.
- Associated conditions like nonimmunologic hydrops fetalis are not typically linked to PBPL.
Abstract:
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.