Cystic Adenomatoid Malformation of the Lung Presenting in Adulthood

Herrero, Yolanda; Pinilla, Inmaculada; Torres, Isabel; Nistal, Manuel; Pardo, Mercedes; Gomez, N.

doi:10.1016/s0003-4975(03)01655-2

Cited by 41 publications

(42 citation statements)

References 7 publications

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“…The lesion can affect both sides of patients with equal frequency and can affect any lung lobe, although it occurs more frequently in the lower lobes and rarely affects more than one lobe (85-95% in only one lobe; Figure 3), being slightly more common in males than in females. (4,15,32) Although CCAM is connected with the tracheobronchial tree and derives its blood supply from the pulmonary circulation, the affected lung area shows reduced vascularization. (15) The incidence of CCAM is approximately 1 case per 10,000-35,000 pregnancies, (4,15,37,38) and CCAM has not been associated with race, age, maternal exposure to any given factor, or genetic factors.…”

Section: Ccammentioning

confidence: 99%

“…(15) The incidence of CCAM is approximately 1 case per 10,000-35,000 pregnancies, (4,15,37,38) and CCAM has not been associated with race, age, maternal exposure to any given factor, or genetic factors. (32) One group of authors (39) conducted a detailed study of CCAMs based on 38 cases and divided CCAMs into 3 types, on the basis of the histological characteristics of the malformations. Initially, CCAMs were classified as types I, II, and III, on the basis of the size of their cysts and histological characteristics, (4,5,15,28,30,31) the frequency for types I, II, and III being 50-70%, 20-40%, and approximately 10%, respectively.…”

Section: Ccammentioning

confidence: 99%

“…Initially, CCAMs were classified as types I, II, and III, on the basis of the size of their cysts and histological characteristics, (4,5,15,28,30,31) the frequency for types I, II, and III being 50-70%, 20-40%, and approximately 10%, respectively. (32) Subsequently, two other types of CCAM, types 0 and IV, were added to the classification. (26) Type I CCAM ( Figure 3) is characterized by a lung mass, generally confined to a lobe, containing a single cyst or multiple cysts over chose to adopt the most common term, i.e., CCAM.…”

Section: Ccammentioning

confidence: 99%

“…(27) It has been shown that CCAMs are hamartomatous lesions, (27) with focal dysplasia and anomalous development, (4,28) and are characterized by a multicystic mass of lung tissue with proliferation of bronchial structures and lung tissue showing aberrant, differentiated architecture, with various degrees of cyst formation. (5,26,(29)(30)(31)(32) The possible mechanisms by which the lesion develops and the exact period of time over which it develops have yet to be determined; however, there is evidence that CCAMs occur between gestational weeks 5 and 22 and result in a wide variety of pathological and radiological presentations. (15) Although there are other theories for the origin of CCAM, some authors believe that it is due to an anomalous development in lung maturation, (29,30) whereas others consider CCAM to represent focal pulmonary dysplasia because skeletal muscle can be identified within the cyst walls and carcinomas can develop.…”

Section: Ccammentioning

confidence: 99%

“…(15,26) In patients with CCAM, there is a broad spectrum of variation in terms of the evolution of the condition, which can be as severe as perinatal death (from hydrops fetalis or pulmonary hypoplasia) or as mild as asymptomatic lesions or spontaneous resolution of symptomatic lesions. (22,27,33,34) The most common symptoms are recurrent infections, (4,27,35) and there have been reports of malignant transformation (to of all CCAMs, (5,15,32,39) and can affect an entire lung or, more rarely, both lungs. (5) The cut-off surface of type III CCAM is firm and generally does not present with cystic lesions; when such lesions are present, they are no larger than 0.5 cm in diameter.…”

Section: Ccammentioning

confidence: 99%

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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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