Current outcome of antenally diagnosed cystic lung disease

Davenport, Mark; Warne, S.A.; Cacciaguerra, Sebastiano; Patel, Shailesh; Greenough, Anne; Nicolaides, Kypros H.

doi:10.1016/j.jpedsurg.2003.12.021

Cited by 287 publications

(273 citation statements)

References 55 publications

(59 reference statements)

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“…2,10 Recommendations have been qualified to include the excision of all cysts occupying more than 25% of one hemithorax. 11 Others have suggested removal if a cyst is greater than 3 cm in diameter or is fluid filled on computed tomography (CT). 12 Some suggest observation alone.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

262

215

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Summary. 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 from benign 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, cystic PPB evolves 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 and multifocal/bilateral lung cysts also characterize PPB. These diagnoses predict that a lung cyst is more likely PPB than a benign congenital cyst. Patients fitting this pattern deserve histologic diagnosis. The genetic basis for this heritable syndrome is unknown but is being actively investigated.

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Section: Introductionmentioning

confidence: 99%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

262

215

View full text Add to dashboard Cite

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“…Failure in vessel occlusion was mainly due to misfocusing. Throughout the experiment, HIFU delivery was controlled by a computer program so that the HIFU energy was emitted when the target was most static in position, although focus-xing failures Adzick reviewed a series of fetal cases having CCAM or BPS, reporting that these lesions are likely to be associated with a prenatal mortality of 100% if compromised by fetal hydrops and managed solely in an expectant way, whereas all the fetuses not associ- However, these procedures are technically quite demanding and frequently invasive to both the mother and fetus 12 . HIFU has been increasingly employed for minimally-or non-invasive treatment in several surgical elds and could be used for compromised fetal lung lesions.…”

Section: Discussionmentioning

confidence: 99%

Innovative Treatment of a Fetal Lung Mass Model Using High-intensity Focused Ultrasound (HIFU)

Nakayama

Kim

Ishiyama

et al. 2011

Showa Univ J Med Sci

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“…[18] The best postnatal management is uncertain for these lesions as conservative management or need for, and timing of, surgical intervention is debated. [19] Based on the current uncertainty, informed consent for the operative intervention must include detailed discussions and the current state of evidence [1].…”

Section: Conclusion Acknowledgementmentioning

confidence: 99%