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• Recent identification of pleuropulmonary blastoma following non-urgent resection of a cystic pulmonary airway malformation.
  Dr Catriona M Middleton, Dr Louise A Selby, Robert I Ross-Russell and Mr Adil Aslam
  Published on: 19 September 2017

• Published on: 19 September 2017

  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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  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 karyotypes from PPB tumours usually contain excessive material from chromosome 8, and an increased incidence of p53 mutations. It has been suggested these mutations differentiate simple CPAM from cases that will later undergo malignant transformation.(3)
  We therefore felt it was valuable to report that malignancy does occur in this group of patients unexpectedly, with significant consequences. There may also be a role for testing genetic aspects of CPAMs; some data suggests p53 mutations within CPAMs may be a useful indicator of risk of PPB.

  1. Cook J, Chitty L, De Coppi P, Ashworth M, Wallis C. The natural history of prenatally diagnosed congenital cystic lung lesions: long-term follow-up of 119 cases. Arch Dis Child 2017;102:798–803.
  2. Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition. Fievet L, Natale C, D’Journo X, Coze S, Dubus J, Guys J, Thomas P, De Lagausie P. J Minim Access Surg. 2015; 11(2): 129–133.
  3. Vargas S, Korpershoek E, Kozakewich H, de Krijger R, Fletcher J, Perez-Atayde A. Cytogenetic and p53 profiles in congenital cystic adenomatoid malformation: insights into its relationship with pleuropulmonary blastoma. Pediatr Dev Pathol. 2006;9(3):190-195

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  Conflict of Interest:
  None declared.

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