Pulmonary cysts in early childhood and the risk of malignancy

John R. Priest, Gretchen M. Williams, D. Ashley Hill, Louis P. Dehner, Adam Jaffé

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169 Scopus citations


Surgery for congenital and early childhood lung cysts is often dictated by symptoms such as respiratory distress, infection or pneumothorax.Asymptomatic cysts present a therapeutic dilemma: surgical intervention and "conservative" observation have advocates. The risk of malignancy in such cysts is considered by some an indication for surgical intervention and is reviewed in this paper. Pleuropulmonary blastoma (PPB) is the most frequent malignancy associated with childhood lung cysts. Although rare, PPB occurs predictably in certain clinical and familial situations. This unique biology of PPB can inform the cyst management decision. The earliest manifestation of PPB is a malignant lung cyst in young children, clinically and radiographically indistinguishable frombenign congenital lung cysts. Histopathologic examination differentiates cystic PPB from the benign cystic variants. Surgical excision of cystic PPB (with or without chemotherapy) cures approximately 85-90%of children. If not excised, cysticPPBevolves to cystic/solid or solid high-grade sarcoma (cure rate 45-60%) by age 2-6 years. Numerous reports of "malignancy in a congenital lung cyst" are now understood as the characteristic progression of cystic PPB. PPB is genetically determined in many cases. Detailed family history may reveal the hallmarks of PPB in the patient or young relatives: a unique constellation of diseases including lung cysts, cystic nephroma, childhood cancers, stromal sex-chord ovarian tumors, seminomas or dysgerminomas, intestinal polyps, thyroid hyperplasias, and hamartomas. Pneumothorax andmultifocal/bilateral lung cysts also characterize PPB. These diagnoses predict that a lung cyst ismore likelyPPBthan a benign congenital cyst.Patients ?tting this pattern deserve histologic diagnosis. The genetic basis for this heritable syndrome is unknown but is being actively investigated.

Original languageEnglish
Pages (from-to)14-30
Number of pages17
JournalPediatric Pulmonology
Issue number1
StatePublished - Jan 1 2009


  • BAC
  • Bronchioloalveolar carcinoma
  • CCAM
  • CPAM
  • Congenital cystic adenomatoid malformation
  • Congenital pulmonary airway malformation
  • PPB
  • Pleuropulmonary blastoma
  • Primary pleuropulmonary synovial sarcoma

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