Type I Pleuropulmonary Blastoma Originating from an Extralobar Sequestration

Pleuropulmonary blastomas (PPBs) represent a rare, malignant neoplasm in infants and children. Despite their overall rarity, they are the most common primary lung malignancy in young children. Typically originating as cystic lesions that are amenable to easy resection, PPBs can progress to solid masses prone to metastases if not identified and treated early enough. In their early cystic form, PPBs are often difficult to distinguish from congenital pulmonary airway malformations based on clinical and radiographic findings. We report on an eight-month-old girl who presented to our institution with a large, right-sided, cystic lung lesion, which had not been apparent on a chest radiograph shortly after birth. After excision of the lesion, pathology demonstrated it to be a type I PPB, which was unique for its apparent extra lobar origin. We review the relevant literature concerning PPBs, as well as the debate surrounding resection of cystic lung lesions in infants and children.