Treatment of Childhood Pleuropulmonary Blastoma

There are no standard treatment options for childhood pleuropulmonary blastoma. Current treatment regimens for these rare tumors have been informed by consensus opinion. The European Cooperative Study Group for Pediatric Rare Tumors within the PARTNER project (Paediatric Rare Tumours Network–European Registry) published comprehensive recommendations for the diagnosis and treatment of pleuropulmonary blastoma in children and adolescents.[1]

Treatment options for childhood pleuropulmonary blastoma include the following:

A complete surgical resection is required for cure.[2]

Data from the International Pleuropulmonary Blastoma Registry and the European Cooperative Study Group for Pediatric Rare Tumors suggest that adjuvant chemotherapy may reduce the risk of recurrence.[3]; [4][Level of evidence C1] Responses to chemotherapy have been reported with agents similar to those used for the treatment of rhabdomyosarcoma.[3-5]

Some general treatment considerations from the International Pleuropulmonary Blastoma Registry, according to subtype, are discussed below.[3,6]