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08.05.2019 | Case Report

Type I regressed pleuropulmonary blastoma in a 10-year-old boy

Indian Journal of Thoracic and Cardiovascular Surgery
Hebah Sameer Al Absi, Sofia Konstantinopoulou, Adel Adel Al Junaibi, Mohammad Fahed Abdullah, Vasudev Sharma, Saeeda Al Marzooqi
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Pleuropulmonary blastoma (PPB) is a rare, malignant tumor of the lung and is the most common primary pulmonary malignancy in children. Here, we report a case of a boy who was diagnosed with type I regressed PPB after being mislabeled with congenital pulmonary malformation. A 10-year-old boy presented to our hospital with a history of worsening dyspnea. Since birth, his clinical status and radiographic images were concerning for congenital lobar emphysema that was managed conservatively. A chest computed tomography (CT) scan confirmed the persistence of a large cystic lesion and a diagnostic and therapeutic cystectomy was performed. Microscopic examination confirmed the presence of PPB type Ir. Patient was managed surgically alone with no added chemotherapy, as there was no overall survival benefit. PPB Ir has an overall favorable clinical outcome. Limited follow-up data are available due to the rarity of the lesion and the overlap with other congenital cystic lung malformations.

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