Childhood pleuropulmonary blastoma: Caution against nonoperative management of congenital lung cysts

Tagge, Edward P.; Mulvihill, Denise; Chandler, John C.; Richardson, Mary S.; Uflacker, Renan; Othersen, H. Biemann

doi:10.1016/s0022-3468(96)90345-0

Cited by 76 publications

(43 citation statements)

References 21 publications

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“…Alguns autores propuseram a ressecção segmental para MACC, mas isso normalmente resulta em ressecção incompleta, bolhas persistentes e a necessidade de lobectomia adicional 25 . [25][26][27] . A observação pode ser uma opção, mas os pacientes devem estar cientes da possibilidade de evolução adversa.…”

Section: Discussionunclassified

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

Giubergia¹,

Barrenechea²,

Siminovich³

et al. 2012

J Pediatr (Rio J)

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Objective: Congenital cystic adenomatoid malformation (CCAM) is the most common surgically resected pulmonary malformation in children. This retrospective study was undertaken to present the experience of 172 CCAM cases in a pediatric hospital. Methods:Published series with a small number of patients reports details of lesions, progress and management. As this study addresses clinical characteristics, progress and surgical procedures in 172 children with CCAM diagnosis, the population includes cases treated and followed up in a pediatric hospital throughout 25 years .Results: Mean age at diagnosis was 48 months (r = 0.03-213), 52% (n = 90) were male. The most common presenting symptoms were respiratory distress in children under 6 months of age (40%) and recurrent pneumonia in older ones (75%; p = 0.001). Lobectomy was the procedure of choice in the majority. All histological types were found: 1 (70%), 2 (24%), 4 (4%), and 0 and 3 (n = 1). A mixed pattern was observed in nine patients. Associated anomalies were found in 47% of children. The most frequent was sequestration (71%), mostly present in CCAM type 2 (p = 0.001). Severe anomalies were mostly related to type 2 (p = 0.008). A pleuropulmonary blastoma and a bronchioloalveolar carcinoma were also observed. Mortality was 5% (n = 9). Risk factors for mortality were respiratory failure ; p = 0.03), sepsis (OR = 9.9 [95%CI 8.2-12]; p = 0.002), respiratory assistance requirements (OR = 9.5 [95%CI 2.3-37]; p = 0.04), and severe associated comorbidities (OR = 3.3 [95%CI 1.2-22]; p = 0.008). Conclusions:Related anomalies were observed in almost half of the population. Due to the possibility of recurrent infection or development of malignancies, surgical resection should be considered when CCAM is diagnosed. Surgical outcome is favorable with manageable complications. J Pediatr (Rio J). 2012;88(2):143-8:Congenital cystic airway malformation, associated malformations, surgical procedures, children. ResumoObjetivo: A malformação adenomatoide cística congênita (MACC) é a malformação pulmonar ressecada cirurgicamente mais comum em crianças. Este estudo retrospectivo foi realizado para apresentar a experiência de 172 casos de MACC em um hospital pediátrico.Métodos: Séries publicadas com um pequeno número de pacientes relatam detalhes de lesões, evolução e tratamento. Como este estudo lida com características clínicas, evolução e procedimentos cirúrgicos em 172 crianças com diagnóstico de MACC, a população inclui casos tratados e acompanhados em um hospital pediátrico ao longo de 25 anos .Resultados: A idade média ao diagnóstico foi de 48 meses (r = 0,03-213), 52% (n = 90) eram do sexo masculino. Os sintomas de apresentação mais comuns foram dificuldade respiratória em crianças com menos de 6 meses de idade (40%) e pneumonia recorrente nas que tinham mais idade (75%; p = 0.001). Lobectomia foi o procedimento de escolha na maioria dos casos. Todos os tipos histológicos foram encontrados: 1 (70%), 2 (24%), 4 (4%) e 0 e 3 (n = 1). Foi observado um padrão misto em nove...

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Section: Discussionunclassified

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

Giubergia¹,

Barrenechea²,

Siminovich³

et al. 2012

J Pediatr (Rio J)

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“…Beyond the neonatal period the main risk is infection [1][2][3][4], which may greatly complicate an eventual resection. The risk of malignant transformation is also real [37][38][39][40] and there is a small risk of spontaneous pneumothorax [41]. Since CCAM does not regress postnatally [42] and since these risks span over decades precluding close follow-up of these patients, simple observation seems hazardous.…”

Section: Discussionmentioning

confidence: 99%

Outcome of the Prenatally Diagnosed Congenital Cystic Adenomatoid Lung Malformation: A Canadian Experience

et al. 2001

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Congenital cystic adenomatoid malformation of the lung (CCAM) is diagnosed by prenatal ultrasonography with an increasing frequency but controversy persists as to its prognosis and prenatal management. Method: A multi-institutional study of cases of CCAM diagnosed antenatally identified by ultrasonographers and by a review of hospital charts. Results: We obtained 48 cases from five centers. We estimate the incidence of CCAM at 1:25,000 to 1:35,000 pregnancies. The incidence of voluntary abortions was 15% (7/48), of spontaneous abortions 2% (1/41) and of postnatal death 10% (4/40). One of the postnatal deaths was from trisomy 18. Of the 7 aborted fetuses, 2 had multiple malformations and 1 had severe hydrops and oligohydramnios; the other 4 had a large mass with mediastinal displacement but without hydrops. When pregnancy was allowed to continue, 56% of the lesions regressed spontaneously, even though one third of these had initial progression. In 17 cases (42%) the mediastinal shift corrected itself, sometimes by simple growth of the fetus but most often by a decrease in the size of the lung mass. In 1 fetus, repeated needle decompressions followed by double-pigtail catheter drainage of large cysts allowed regression of hydrops. Despite this, neonatal death occurred from pulmonary hypoplasia. Conclusion: CCAM can lead to fetal or neonatal demise from hydrops, lung hypoplasia, prematurity or severe associated malformations, but has a good prognosis in the majority of cases.

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“…Lesions thought to be CCAM must be followed to involution or at least yearly reimaged if not removed. 12 Type 2 lesions may be characteristic and allow a correct diagnosis since both the cystic and solid components can be visualized. Type 3 is usually a large lesion often opacifying the hemithorax and associated with a pleural effusion.…”

Section: Denouement and Discussionmentioning

confidence: 99%

Neonatal pleuropulmonary blastoma, type 1

Herman

Siegel

2007

J Perinatol

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Childhood pleuropulmonary blastoma: Caution against nonoperative management of congenital lung cysts

Cited by 76 publications

References 21 publications

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

Outcome of the Prenatally Diagnosed Congenital Cystic Adenomatoid Lung Malformation: A Canadian Experience

Neonatal pleuropulmonary blastoma, type 1

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