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The natural history of prenatally diagnosed congenital cystic lung lesions: long-term follow-up of 119 cases
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  • Published on:
    Recent identification of pleuropulmonary blastoma following non-urgent resection of a cystic pulmonary airway malformation.
    • Dr Catriona M Middleton, Paediatric Specialty Registrar Addenbrookes Hospital, Cambridge
    • Other Contributors:
      • Dr Louise A Selby, Paediatric Respiratory Specialty Registrar
      • Robert I Ross-Russell, Paediatric Respiratory Consultant
      • Mr Adil Aslam, Paediatric surgeon

    We read with interest the recent paper by Cook et al(1) reporting their experience with 119 cases of cystic pulmonary airway malformation (CPAM); in which no reported cases showed malignant change. The potential for malignant transformation of CPAM is well-described but extremely rare. Type 1 can predispose to mucinous bronchiolo-alveolar carcinoma in adults, type 2 are associated with pleuropulmonary blastoma (PPB)(2). We describe a case of PPB diagnosed histologically following non-urgent resection of a CPAM.
    A 10-month old boy was routinely referred by primary care with episodic wheeze and shortness of breath. He was thriving with no clinical stigmata nor symptoms of malignancy, and no relevant family history.
    Examination noted reduced air entry to the left lung; chest x-ray showed extensive left sided hyperlucency with mediastinal shift. Urgent chest CT with contrast demonstrated a very large multi-septated cystic malformation arising in the left lower lobe, with no systemic arterial supply. He was referred to the paediatric thoracic surgeons.
    Four months later he had reduced exercise tolerance and one brief admission for pneumonia. Surgical excision took place one year after CT imaging. Resection was uncomplicated and subsequent histological identification of a type 2 CPAM with PPB was unexpected. There were nodules containing malignant spindle epithelioid cells and he has since commenced chemotherapy.
    Previous papers have shown that karyoty...

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    Conflict of Interest:
    None declared.