Are Bilateral Fetal Lung Masses Double Trouble?

Lipshutz, Gerald S.; Lopoo, John B.; Jennings, Russel W.; Farrell, Jody A.; Harrison, Michael R.; Albanese, Craig T.

doi:10.1159/000020956

Cited by 5 publications

(3 citation statements)

References 13 publications

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“…Among 223 IPPBR cases, bilateral cysts were found in 23 (10%) and unilateral multifocal cysts in 11 (5%). This finding may help differentiate PPB from CPAM, which tends to be unilateral (98% of cases 40,59 ) and confined to one lobe (95% of cases 40 ). The extent of cystic change in PPB may be highly asymmetric between involved areas of lung.…”

Section: Pleuropulmonary Blastoma (Ppb)mentioning

confidence: 93%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

272

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: Pleuropulmonary Blastoma (Ppb)mentioning

confidence: 93%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

272

215

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“…The finding of bilateral lung hyperechogenicity is very rare but can be caused with tracheal atresia and, therefore, can be associated with a poor prognosis [38,45]; however, cases with bilateral lesions, such as CCAM, surviving to term, have been published. Lipshutz et al published a series of three antenatally identified bilateral lesions two of whom survived to term [47].…”

Section: Antenatal Ultrasoundmentioning

confidence: 99%

Bronchopulmonary foregut malformations: embryology, radiology and quandary

Barnes

Pilling

2003

European Radiology

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Bronchopulmonary foregut malformations (BPFM) are a heterogeneous group of pulmonary developmental anomalies that present at varying ages and with overlapping symptoms, signs and radiology. This article discusses the embryology of these lesions with reference to possible common origins and the link between aetiology and radiological appearance. The radiology of each lesion, both antenatally and postnatally, is described and illustrated. A number of quandaries exist in the prediction of prognosis and subsequent treatment of BPFM. We discuss the radiological features that may help to elucidate an individual prognosis and aid in the planning of treatment. The treatment options available for BPFM are briefly discussed. Finally, the link between BPFM, in particular cystic adenomatoid malformations and malignancy, is discussed. We aim to provide a comprehensive overview of the embryology, radiology, prognosis and treatment highlighting contentious issues of BPFM.

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“…Lesions are typically unilateral, unilobular, and noncompressible, leading to surgical resection. Rarely are CCAMs bilateral or involve the entire lung [8]. These cystic lesions with variable quantities of proliferating bronchial structures compress surrounding lung parenchyma and interfere with normal alveolar development.…”

mentioning

confidence: 99%

Prenatal Therapy for Thoracic and Mediastinal Lesions

2003

Self Cite

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Most prenatally diagnosed lung lesions can be managed successfully during the neonatal period. Prenatal imaging and experimental models have provided a comprehensive understanding of the pathophysiology, natural history, and prognosis of intrathoracic and mediastinal lesions. Clinical experience has demonstrated that progression to nonimmune hydrops fetalis and pulmonary hypoplasia is a harbinger of fetal or neonatal demise. Advances in fetal anesthesia, tocolysis, and surgical techniques have made fetal surgery a viable in utero option to ameliorate life-threatening masses. Congenital cystic adenomatoid malformation, bronchopulmonary sequestration, and congenital hydrothorax are the most common abnormalities amenable to surgical intervention. The natural history, evaluation, and treatment of intrathoracic and mediastinal lesions are discussed.

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Are Bilateral Fetal Lung Masses Double Trouble?

Cited by 5 publications

References 13 publications

Pulmonary cysts in early childhood and the risk of malignancy

Pulmonary cysts in early childhood and the risk of malignancy

Bronchopulmonary foregut malformations: embryology, radiology and quandary

Prenatal Therapy for Thoracic and Mediastinal Lesions

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