Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma?

Nasr, Ahmed; Himidan, Sharifa; Pastor, Aimee C.; Taylor, Glenn; Kim, Peter C.W.

doi:10.1016/j.jpedsurg.2010.02.067

Cited by 118 publications

(71 citation statements)

References 19 publications

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“…Fewer than 500 cases have been reported, with an estimated incidence of 1 in 250,000 live births [2]. In 25-38% of cases, it is associated with other dysplastic or neoplastic anomalies in either the patient or family members [1].…”

Section: Discussionmentioning

confidence: 99%

“…PPB presents a diagnostic challenge since it is difficult to distinguish from benign lung lesions either prenatally or in early childhood [2,3,4]. Thus, the diagnosis is most often only made by histology.…”

Section: Discussionmentioning

confidence: 99%

“…Three types of PPB are described based on pathology - purely cystic (type I), mixed (type II) and solid (type III) [1]. In a recent report by Nasr et al [2], all 3 resected type I PPBs were deemed clinically and radiologically indistinguishable from CPAMs. A solid type III tumor is more pathognomonic but typically presents in older children [2].…”

Section: Discussionmentioning

confidence: 99%

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Pleuropulmonary Blastoma in a Neonate Diagnosed Prenatally as Congenital Pulmonary Airway Malformation

Coleman¹,

Kline‐Fath

Stanek³

et al. 2015

Fetal Diagn Ther

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Pleuropulmonary blastoma is an extremely rare lung neoplasm exclusive to children under 5 years of age. It presents a diagnostic challenge both prenatally and in early childhood due to its similarity to benign lung cysts, which are managed differently. We present the first case, to our knowledge, of a neonate with pleuropulmonary blastoma and myelomeningocele, though prenatally diagnosed as a congenital pulmonary airway malformation. We detail the prenatal imaging that facilitated counseling and delivery management in addition to the correlating postnatal imaging.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Pleuropulmonary Blastoma in a Neonate Diagnosed Prenatally as Congenital Pulmonary Airway Malformation

Coleman¹,

Kline‐Fath

Stanek³

et al. 2015

Fetal Diagn Ther

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“…(4,5,8,15,20,33,(36)(37)(38) Recently, one group of authors studied 129 cases of CCAM and found that the incidence of pleuropulmonary blastoma among the 54 patients who underwent surgery was 4%. (43) Although such tumors are rare, over 8% are associated with CCAM. In symptomatic patients, there is consensus that the lesion must be resected, generally by means of pulmonary lobectomy, which avoids residual disease and recurrence in the remaining tissue.…”

Section: Ccammentioning

confidence: 99%

Malformações pulmonares congênitas

2011

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Congenital lung malformations are rare and vary widely in their clinical presentation and severity, depending mostly on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Individuals with congenital lung malformations can present with respiratory symptoms at birth or can remain asymptomatic for long periods. Recently, there has been an increase in the early diagnosis of these malformations, a change that is attributable to the routine use of prenatal ultrasound. The clinical manifestation of these malformations varies from respiratory distress in the immediate postnatal period to an incidental finding on chest X-rays. Early diagnosis and prompt treatment offer the possibility of absolutely normal lung development. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these diseases is unpredictable. The management of these lesions depends on the type of malformation and symptoms. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. Lobectomy is the procedure of choice and yields excellent long-term results. This article describes the principal congenital lung malformations, their diagnosis, and the controversies regarding treatment.Keywords: Cystic adenomatoid malformation of lung, congenital; Bronchopulmonary sequestration; Pulmonary surgical procedures; Diagnosis. ResumoAs malformações congênitas do pulmão são raras e variam muito na sua forma de apresentação clínica e gravidade, dependendo principalmente do grau de envolvimento pulmonar e de sua localização na cavidade torácica. Elas podem se manifestar em qualquer idade e podem ser fonte de importante morbidade e mortalidade em lactentes e crianças. Os indivíduos com malformações congênitas do pulmão podem apresentar sintomas respiratórios ao nascimento, enquanto outros podem permanecer assintomáticos por longos períodos. Atualmente, com o uso rotineiro da ultrassonografia pré-natal, vem ocorrendo um aumento no diagnóstico mais precoce dessas malformações. A manifestação clínica dessas malformações varia desde uma disfunção respiratória pós-natal imediata a um achado acidental na radiografia de tórax. O diagnóstico precoce e o tratamento imediato oferecem a possibilidade de um desenvolvimento pulmonar absolutamente normal. Quando assintomáticos, a conduta para o tratamento dos pacientes com malformações pulmonares ainda é controversa, uma vez que o prognóstico dessas afecções é imprevisível. O manejo dessas lesões depende do tipo de malformação e de sintomas. Devido ao risco de complicação, a maioria dos autores sugere a ressecção da lesão no momento em que essa é identificada. A lobectomia é o procedimento de escolha, fornecendo excelentes resultados a longo prazo. Este artigo descreve as principais malformações pulmonares congênitas, seu diagnóstico e controvérsias quanto o tratamento. (5) is covered by ...

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“…Worryingly, in a large Canadian study, CTMs which turned out to be or became a pleuropulmonary blastoma (admittedly a rare event) were indistinguishable from benign lesions [6]. And finally, even after complete resection of a CTM, the risk of malignancy elsewhere in the lungs remains.…”

mentioning

confidence: 99%