Congenital pulmonary airway malformation (CPAM) is one of the most common lung lesions detected prenatally. Despite the research efforts made in the past few years, controversy and lack of clarity in the literature still exist regarding nomenclature, classification, pathogenesis and the management of CPAM. Therefore, it is of greatest importance to delineate the natural history of CPAMs and to create a consensus to guide the management and follow-up of these lesions. This review will focus on classification systems, highlighting the most recent advancements in pathogenesis, and current practice in the prenatal diagnosis of CPAM. Strategies of prenatal management and postnatal management will be reviewed. Long-term follow-up, including lung cancer risk, is discussed and an outcome perspective is presented.

Keywords:
Congenital malformations, Lung development, Macrocystic pulmonary lesion, Microcystic pulmonary lesion, Thoracoscopic lobectomy, Lung cancer risk
1.
Stocker JT, Madewell JE, Drake RM: Congenital cystic adenomatoid malformation of the lung: classification and morphologic spectrum. Hum Pathol 1977;8:155-171.
2.
Adzick NS, Harrison MR, Glick PL, Golbus MS, Anderson RL, Mahony BS, et al: Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg 1985;20:483-488.
3.
Ben-Ishay O, Nicksa GA, Wilson JM, Buchmiller TL: Management of giant congenital pulmonary airway malformations requiring pneumonectomy. Ann Thorac Surg 2012;94:1073-1078.
4.
Laberge JM, Puligandla P, Flageole H: Asymptomatic congenital lung malformations. Semin Pediatr Surg 2005;14:16-33.
5.
Cass DL, Olutoye OO, Cassady CI, Moise KJ, Johnson A, Papanna R, et al: Prenatal diagnosis and outcome of fetal lung masses. J Pediatr Surg 2011;46:292-298.
6.
Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C, et al: Antenatal and postnatal management of congenital cystic adenomatoid malformation. Paediatr Respir Rev 2012;13:162-170.
7.
Nagata K, Masumoto K, Tesiba R, Esumi G, Tsukimori K, Norio W, et al: Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lung. Pediatr Surg Int 2009;25:753-757.
8.
Langston C: New concepts in the pathology of congenital lung malformations. Semin Pediatr Surg 2003;12:17-37.
9.
Cass DL, Crombleholme TM, Howell LJ, Stafford PW, Ruchelli ED, Adzick NS: Cystic lung lesions with systemic arterial blood supply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg 1997;32:986-990.
10.
Ch'In KY, Tang MY: Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol 1949;48:221-229.
11.
Stocker JT: Congenital and developmental diseases; in Dail DH, Hammar SP (eds): Pulmonary Pathology, ed 3. New York, Springer, 2008, pp 154-180.
12.
Newman B: Congenital bronchopulmonary foregut malformations: concepts and controversies. Pediatr Radiol 2006;36:773-791.
13.
Morotti RA, Cangiarella J, Gutierrez MC, Jagirdar J, Askin F, Singh G, et al: Congenital cystic adenomatoid malformation of the lung (CCAM): evaluation of the cellular components. Hum Pathol 1999;30:618-625.
14.
Morotti RA, Gutierrez MC, Askin F, Profitt SA, Wert SE, Whitsett JA, et al: Expression of thyroid transcription factor-1 in congenital cystic adenomatoid malformation of the lung. Pediatr Dev Pathol 2000;3:455-461.
15.
Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ: Fetal lung lesions: management and outcome. Am J Obstet Gynecol 1998;179:884-889.
16.
Correia-Pinto J, Gonzaga S, Huang Y, Rottier R: Congenital lung lesions - underlying molecular mechanisms. Semin Pediatr Surg 2010;19:171-179.
17.
Wilson RD, Hedrick HL, Liechty KW, Flake AW, Johnson MP, Bebbington M, et al: Cystic adenomatoid malformation of the lung: review of genetics, prenatal diagnosis, and in utero treatment. Am J Med Genet A 2006;140:151-155.
18.
Bush A: Congenital lung disease: a plea for clear thinking and clear nomenclature. Pediatr Pulmonol 2001;32:328-337.
19.
Ramos SG, Barbosa GH, Tavora FR, Jeudy J, Torres LA, Tone LG, et al: Bronchioloalveolar carcinoma arising in a congenital pulmonary airway malformation in a child: case report with an update of this association. J Pediatr Surg 2007;42:E1-E4.
20.
Williams HJ, Johnson KJ: Imaging of congenital cystic lung lesions. Paediatr Respir Rev 2002;3:120-127.
21.
Vu L, Tsao K, Lee H, Nobuhara K, Farmer D, Harrison M, et al: Characteristics of congenital cystic adenomatoid malformations associated with nonimmune hydrops and outcome. J Pediatr Surg 2007;42:1351-1356.
22.
Rosado-de-Christenson ML, Stocker JT: Congenital cystic adenomatoid malformation. Radiographics 1991;11:865-886.
23.
Raychaudhuri P, Pasupati A, James A, Whitehead B, Kumar R: Prospective study of antenatally diagnosed congenital cystic adenomatoid malformations. Pediatr Surg Int 2011;27:1159-1164.
24.
van Koningsbruggen S, Ahrens F, Brockmann M, Michalk D, Rietschel E: Congenital cystic adenomatoid malformation type 4. Pediatr Pulmonol 2001;32:471-475.
25.
Joshi S, Kotecha S: Lung growth and development. Early Hum Dev 2007;83:789-794.
26.
Groenman F, Unger S, Post M: The molecular basis for abnormal human lung development. Biol Neonate 2005;87:164-177.
27.
Perl AK, Whitsett JA: Molecular mechanisms controlling lung morphogenesis. Clin Genet 1999;56:14-27.
28.
Cass DL, Quinn TM, Yang EY, Liechty KW, Crombleholme TM, Flake AW, et al: Increased cell proliferation and decreased apoptosis characterize congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 1998;33:1043-1046; discussion 1047.
29.
Simonet WS, DeRose ML, Bucay N, Nguyen HQ, Wert SE, Zhou L, et al: Pulmonary malformation in transgenic mice expressing human keratinocyte growth factor in the lung. Proc Natl Acad Sci USA 1995;92:12461-12465.
30.
Liechty KW, Crombleholme TM, Quinn TM, Cass DL, Flake AW, Adzick NS: Elevated platelet-derived growth factor-B in congenital cystic adenomatoid malformations requiring fetal resection. J Pediatr Surg 1999;34:805-809; discussion 809-810.
31.
Fromont-Hankard G, Philippe-Chomette P, Delezoide AL, Nessmann C, Aigrain Y, Peuchmaur M: Glial cell-derived neurotrophic factor expression in normal human lung and congenital cystic adenomatoid malformation. Arch Pathol Lab Med 2002;126:432-436.
32.
Volpe MV, Pham L, Lessin M, Ralston SJ, Bhan I, Cutz E, et al: Expression of Hoxb-5 during human lung development and in congenital lung malformations. Birth Defects Res A Clin Mol Teratol 2003;67:550-556.
33.
Jancelewicz T, Nobuhara K, Hawgood S: Laser microdissection allows detection of abnormal gene expression in cystic adenomatoid malformation of the lung. J Pediatr Surg 2008;43:1044-1051.
34.
Wagner AJ, Stumbaugh A, Tigue Z, Edmondson J, Paquet AC, Farmer DL, et al: Genetic analysis of congenital cystic adenomatoid malformation reveals a novel pulmonary gene: fatty acid binding protein-7 (brain type). Pediatr Res 2008;64:11-16.
35.
Volpe MV, Chung E, Ulm JP, Gilchrist BF, Ralston S, Wang KT, et al: Aberrant cell adhesion molecule expression in human bronchopulmonary sequestration and congenital cystic adenomatoid malformation. Am J Physiol Lung Cell Mol Physiol 2009;297:L143-L152.
36.
Gontan C, de Munck A, Vermeij M, Grosveld F, Tibboel D, Rottier R: Sox2 is important for two crucial processes in lung development: branching morphogenesis and epithelial cell differentiation. Dev Biol 2008;317:296-309.
37.
Schilders K, Ochieng JK, van de Ven CP, Gontan C, Tibboel D, Rottier RJ: Role of SOX2 in foregut development in relation to congenital abnormalities. World J Med Genet 2014;4:94-104.
38.
Gonzaga S, Henriques-Coelho T, Davey M, Zoltick PW, Leite-Moreira AF, Correia-Pinto J, et al: Cystic adenomatoid malformations are induced by localized FGF10 overexpression in fetal rat lung. Am J Respir Cell Mol Biol 2008;39:346-355.
39.
Griffin N, Devaraj A, Goldstraw P, Bush A, Nicholson AG, Padley S: CT and histopathological correlation of congenital cystic pulmonary lesions: a common pathogenesis? Clin Radiol 2008;63:995-1005.
40.
Breysem L, Bosmans H, Dymarkowski S, Schoubroeck DV, Witters I, Deprest J, et al: The value of fast MR imaging as an adjunct to ultrasound in prenatal diagnosis. Eur Radiol 2003;13:1538-1548.
41.
Tsai AY, Liechty KW, Hedrick HL, Bebbington M, Wilson RD, Johnson MP, et al: Outcomes after postnatal resection of prenatally diagnosed asymptomatic cystic lung lesions. J Pediatr Surg 2008;43:513-517.
42.
Farrugia MK, Raza SA, Gould S, Lakhoo K: Congenital lung lesions: classification and concordance of radiological appearance and surgical pathology. Pediatr Surg Int 2008;24:987-991.
43.
Makhija Z, Moir CR, Allen MS, Cassivi SD, Deschamps C, Nichols F 3rd, et al: Surgical management of congenital cystic lung malformations in older patients. Ann Thorac Surg 2011;91:1568-1573; discussion 1573.
44.
Marshall KW, Blane CE, Teitelbaum DH, van Leeuwen K: Congenital cystic adenomatoid malformation: impact of prenatal diagnosis and changing strategies in the treatment of the asymptomatic patient. AJR Am J Roentgenol 2000;175:1551-1554.
45.
Alamo L, Gudinchet F, Reinberg O, Vial Y, Francini K, Osterheld MC, et al: Prenatal diagnosis of congenital lung malformations. Pediatr Radiol 2012;42:273-283.
46.
Kline-Fath B, Bitters C: Prenatal imaging of fetal lung lesions: magnetic resonance imaging complements ultrasound. J Radiol Nurs 2010;29:3-9.
47.
Epelman M, Kreiger PA, Servaes S, Victoria T, Hellinger JC: Current imaging of prenatally diagnosed congenital lung lesions. Semin Ultrasound CT MR 2010;31:141-157.
48.
Liu YP, Chen CP, Shih SL, Chen YF, Yang FS, Chen SC: Fetal cystic lung lesions: evaluation with magnetic resonance imaging. Pediatr Pulmonol 2010;45:592-600.
49.
Shanmugam G, MacArthur K, Pollock JC: Congenital lung malformations - antenatal and postnatal evaluation and management. Eur J Cardiothorac Surg 2005;27:45-52.
50.
Sauvat F, Michel JL, Benachi A, Emond S, Revillon Y: Management of asymptomatic neonatal cystic adenomatoid malformations. J Pediatr Surg 2003;38:548-552.
51.
Cavoretto P, Molina F, Poggi S, Davenport M, Nicolaides KH: Prenatal diagnosis and outcome of echogenic fetal lung lesions. Ultrasound Obstet Gynecol 2008;32:769-783.
52.
Crombleholme TM, Coleman B, Hedrick H, Liechty K, Howell L, Flake AW, et al: Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg 2002;37:331-338.
53.
Azizkhan RG, Crombleholme TM: Congenital cystic lung disease: contemporary antenatal and postnatal management. Pediatr Surg Int 2008;24:643-657.
54.
Eber E: Antenatal diagnosis of congenital thoracic malformations: early surgery, late surgery, or no surgery? Semin Respir Crit Care Med 2007;28:355-366.
55.
Cass DL, Olutoye OO, Ayres NA, Moise KJ Jr, Altman CA, Johnson A, et al: Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors. J Pediatr Surg 2012;47:40-45.
56.
Ankermann T, Oppermann HC, Engler S, Leuschner I, von Kaisenberg CS: Congenital masses of the lung, cystic adenomatoid malformation versus congenital lobar emphysema. J Ultrasound Med 2004;23:1379-1384.
57.
Laje P, Liechty KW: Postnatal management and outcome of prenatally diagnosed lung lesions. Prenat Diagn 2008;28:612-618.
58.
Bunduki V, Ruano R, da Silva MM, Miguelez J, Miyadahira S, Maksoud JG, et al: Prognostic factors associated with congenital cystic adenomatoid malformation of the lung. Prenat Diagn 2000;20:459-464.
59.
Blau H, Barak A, Karmazyn B, Mussaffi H, Ben Ari J, Schoenfeld T, et al: Postnatal management of resolving fetal lung lesions. Pediatrics 2002;109:105-108.
60.
Bush A, Hogg J, Chitty LS: Cystic lung lesions - prenatal diagnosis and management. Prenat Diagn 2008;28:604-611.
61.
Chowdhury MM, Chakraborty S: Imaging of congenital lung malformations. Semin Pediatr Surg 2015;24:168-175.
62.
Kongstad T, Buchvald F, Brenoe J, Petersen BL, Tabor A, Nielsen KG: Radiology, histology and short-term outcome of asymptomatic congenital thoracic malformations. Acta Paediatr 2012;101:155-158.
63.
Witlox RS, Lopriore E, Oepkes D: Prenatal interventions for fetal lung lesions. Prenat Diagn 2011;31:628-636.
64.
Knox EM, Kilby MD, Martin WL, Khan KS: In-utero pulmonary drainage in the management of primary hydrothorax and congenital cystic lung lesion: a systematic review. Ultrasound Obstet Gynecol 2006;28:726-734.
65.
Loh KC, Jelin E, Hirose S, Feldstein V, Goldstein R, Lee H: Microcystic congenital pulmonary airway malformation with hydrops fetalis: steroids vs open fetal resection. J Pediatr Surg 2012;47:36-39.
66.
Tsao K, Hawgood S, Vu L, Hirose S, Sydorak R, Albanese CT, et al: Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy. J Pediatr Surg 2003;38:508-510.
67.
Curran PF, Jelin EB, Rand L, Hirose S, Feldstein VA, Goldstein RB, et al: Prenatal steroids for microcystic congenital cystic adenomatoid malformations. J Pediatr Surg 2010;45:145-150.
68.
Adzick NS: Management of fetal lung lesions. Clin Perinatol 2003;30:481-492.
69.
Adzick NS: Open fetal surgery for life-threatening fetal anomalies. Semin Fetal Neonatal Med 2010;15:1-8.
70.
Higby K, Melendez BA, Heiman HS: Spontaneous resolution of nonimmune hydrops in a fetus with a cystic adenomatoid malformation. J Perinatol 1998;18:308-310.
71.
Peranteau WH, Wilson RD, Liechty KW, Johnson MP, Bebbington MW, Hedrick HL, et al: Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival. Fetal Diagn Ther 2007;22:365-371.
72.
Morris LM, Lim FY, Livingston JC, Polzin WJ, Crombleholme TM: High-risk fetal congenital pulmonary airway malformations have a variable response to steroids. J Pediatr Surg 2009;44:60-65.
73.
Derderian S, Coleman A, Jeanty C, Lim F, Shaaban A, Farrell J, et al: Favorable outcomes in high-risk congenital pulmonary airway malformations treated with multiple courses of maternal betamethasone. J Pediatr Surg 2015;50:515-518.
74.
San Feliciano L, Remesal A, Isidoro-García M, Ludeña D: Dexamethasone and betamethasone for prenatal lung maturation: differences in vascular endothelial growth factor expression and alveolarization in rats. Neonatology 2011;100:105-110.
75.
Remesal A, San Feliciano L, Isidoro-García M, Ludeña D: Effects of antenatal betamethasone and dexamethasone on the lung expression of vascular endothelial growth factor and alveolarization in newborn rats exposed to acute hypoxia and recovered in normoxia or hyperoxia. Neonatology 2010;98:313-320.
76.
Schrey S, Kelly EN, Langer JC, Davies GA, Windrim R, Seaward PG, et al: Fetal thoracoamniotic shunting for large macrocystic congenital cystic adenomatoid malformations of the lung. Ultrasound Obstet Gynecol 2012;39:515-520.
77.
Wilson RD: In utero therapy for fetal thoracic abnormalities. Prenat Diagn 2008;28:619-625.
78.
Wilson RD, Baxter JK, Johnson MP, King M, Kasperski S, Crombleholme TM, et al: Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations. Fetal Diagn Ther 2004;19:413-420.
79.
Merchant AM, Peranteau W, Wilson RD, Johnson MP, Bebbington MW, Hedrick HL, et al: Postnatal chest wall deformities after fetal thoracoamniotic shunting for congenital cystic adenomatoid malformation. Fetal Diagn Ther 2007;22:435-439.
80.
Matsuoka K, Hayashi S, Urano F, Zhu LJ, Okita H, Sago H, et al: Squamous metaplasia in the cyst epithelium of type 1 congenital pulmonary airway malformation after thoracoamniotic shunt placement. Hum Pathol 2012;43:1413-1717.
81.
Liechty KW, Crombleholme TM: Management of fetal airway obstruction. Semin Perinatol 1999;23:496-506.
82.
Bouchard S, Johnson MP, Flake AW, Howell LJ, Myers LB, Adzick NS, et al: The EXIT procedure: experience and outcome in 31 cases. J Pediatr Surg 2002;37:418-426.
83.
Hedrick HL: Ex utero intrapartum therapy. Semin Pediatr Surg 2003;12:190-195.
84.
Crombleholme T, Albanese C: The fetus with airway obstruction; in: The Unborn Patient: The Art and Science of Fetal Therapy. Philadelphia, Saunders, 2001, pp 357-372.
85.
Harrison MR, Adzick NS, Flake AW, VanderWall KJ, Bealer JF, Howell LJ, et al: Correction of congenital diaphragmatic hernia in utero VIII: response of the hypoplastic lung to tracheal occlusion. J Pediatr Surg 1996;31:1339-1348.
86.
Hirose S, Harrison MR: The ex utero intrapartum treatment (EXIT) procedure. Semin Neonatol 2003;8:207-214.
87.
Marwan A, Crombleholme TM: The EXIT procedure: principles, pitfalls, and progress. Semin Pediatr Surg 2006;15:107-115.
88.
Liechty KW: Ex-utero intrapartum therapy. Semin Fetal Neonatal Med 2010;15:34-39.
89.
Hedrick HL, Flake AW, Crombleholme TM, Howell LJ, Johnson MP, Wilson RD, et al: The ex utero intrapartum therapy procedure for high-risk fetal lung lesions. J Pediatr Surg 2005;40:1038-1043; discussion 1044.
90.
Cass DL, Olutoye OO, Cassady CI, Zamora IJ, Ivey RT, Ayres NA, et al: EXIT-to-resection for fetuses with large lung masses and persistent mediastinal compression near birth. J Pediatr Surg 2013;48:138-144.
91.
Mann S, Wilson RD, Bebbington MW, Adzick NS, Johnson MP: Antenatal diagnosis and management of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med 2007;12:477-481.
92.
Khalek N, Johnson MP: Management of prenatally diagnosed lung lesions. Semin Pediatr Surg 2013;22:24-29.
93.
Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C, et al: Antenatal and postnatal management of congenital cystic adenomatoid malformation. Paediatr Respir Rev 2012;13:162-171.
94.
Lo AY, Jones S: Lack of consensus among Canadian pediatric surgeons regarding the management of congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 2008;43:797-799.
95.
Calvert JK, Lakhoo K: Antenatally suspected congenital cystic adenomatoid malformation of the lung: postnatal investigation and timing of surgery. J Pediatr Surg 2007;42:411-414.
96.
Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP: Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer 1997;80:147-161.
97.
van Leeuwen K, Teitelbaum DH, Hirschl RB, Austin E, Adelman SH, Polley TZ, et al: Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications, and natural history. J Pediatr Surg 1999;34:794-798; discussion 798-799.
98.
Aspirot A, Puligandla PS, Bouchard S, Su W, Flageole H, Laberge JM: A contemporary evaluation of surgical outcome in neonates and infants undergoing lung resection. J Pediatr Surg 2008;43:508-512.
99.
Truitt AK, Carr SR, Cassese J, Kurkchubasche AG, Tracy TF Jr, Luks FI: Perinatal management of congenital cystic lung lesions in the age of minimally invasive surgery. J Pediatr Surg 2006;41:893-896.
100.
Stanton M, Njere I, Ade-Ajayi N, Patel S, Davenport M: Systematic review and meta-analysis of the postnatal management of congenital cystic lung lesions. J Pediatr Surg 2009;44:1027-1033.
101.
Bagolan P, Nahom A, Giorlandino C, Trucchi A, Bilancioni E, Inserra A, et al: Cystic adenomatoid malformation of the lung: clinical evolution and management. Eur J Pediatr 1999;158:879-882.
102.
Dehner LP: Pleuropulmonary blastoma is THE pulmonary blastoma of childhood. Semin Diagnostic Pathol 1994;11:144-151.
103.
Aziz D, Langer JC, Tuuha SE, Ryan G, Ein SH, Kim PC: Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not? J Pediatr Surg 2004;39:329-334; discussion 329-334.
104.
Priest JR, Williams GM, Hill DA, Dehner LP, Jaffe A: Pulmonary cysts in early childhood and the risk of malignancy. Pediatr Pulmonol 2009;44:14-30.
105.
Komori K, Kamagata S, Hirobe S, Toma M, Okumura K, Muto M, et al: Radionuclide imaging study of long-term pulmonary function after lobectomy in children with congenital cystic lung disease. J Pediatr Surg 2009;44:2096-2100.
106.
Zach MS, Eber E: Adult outcome of congenital lower respiratory tract malformations. Thorax 2001;56:65-72.
107.
Hill DA, Dehner LP: A cautionary note about congenital cystic adenomatoid malformation (CCAM) type 4. Am J Surg Pathol 2004;28:554-555; author reply 555.
108.
Parikh D, Samuel M: Congenital cystic lung lesions: is surgical resection essential? Pediatr Pulmonol 2005;40:533-537.
109.
Thurlbeck WM: Postnatal human lung growth. Thorax 1982;37:564-571.
110.
Werner HA, Pirie GE, Nadel HR, Fleisher AG, LeBlanc JG: Lung volumes, mechanics, and perfusion after pulmonary resection in infancy. J Thorac Cardiovasc Surg 1993;105:737-742.
111.
Burri PH: Structural aspects of postnatal lung development - alveolar formation and growth. Biol Neonate 2006;89:313-322.
112.
Merkus PJ, ten Have-Opbroek AA, Quanjer PH: Human lung growth: a review. Pediatr Pulmonol 1996;21:383-397.
113.
Keijzer R, Chiu PP, Ratjen F, Langer JC: Pulmonary function after early vs late lobectomy during childhood: a preliminary study. J Pediatr Surg 2009;44:893-895.
114.
Hancock BJ, Di Lorenzo M, Youssef S, Yazbeck S, Marcotte JE, Collin PP: Childhood primary pulmonary neoplasms. J Pediatr Surg 1993;28:1133-1136.
115.
Nasr A, Bass J: Thoracoscopic vs open resection of congenital lung lesions: a meta-analysis. J Pediatr Surg 2012;47:857-861.
116.
Bratu I, Flageole H, Chen MF, Di Lorenzo M, Yazbeck S, Laberge JM: The multiple facets of pulmonary sequestration. J Pediatr Surg 2001;36:784-790.
117.
Davenport M, Warne SA, Cacciaguerra S, Patel S, Greenough A, Nicolaides K: Current outcome of antenally diagnosed cystic lung disease. J Pediatr Surg 2004;39:549-556.
118.
Papagiannopoulos KA, Sheppard M, Bush AP, Goldstraw P: Pleuropulmonary blastoma: is prophylactic resection of congenital lung cysts effective? Ann Thorac Surg 2001;72:604-605.
119.
Muller CO, Berrebi D, Kheniche A, Bonnard A: Is radical lobectomy required in congenital cystic adenomatoid malformation? J Pediatr Surg 2012;47:642-645.
120.
Vu LT, Farmer DL, Nobuhara KK, Miniati D, Lee H: Thoracoscopic versus open resection for congenital cystic adenomatoid malformations of the lung. J Pediatr Surg 2008;43:35-39.
121.
Khosa JK, Leong SL, Borzi PA: Congenital cystic adenomatoid malformation of the lung: indications and timing of surgery. Pediatr Surg Int 2004;20:505-508.
122.
Kim HK, Choi YS, Kim K, Shim YM, Ku GW, Ahn KM, et al: Treatment of congenital cystic adenomatoid malformation: should lobectomy always be performed? Ann Thorac Surg 2008;86:249-253.
123.
Schneider DT: Rare tumors in children and adolescents. Heidelberg, Springer, 2012.
124.
Nasr A, Himidan S, Pastor AC, Taylor G, Kim PC: Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma? J Pediatr Surg 2010;45:1086-1089.
125.
Priest JR, Hill DA, Williams GM, Moertel CL, Messinger Y, Finkelstein MJ, et al: Type I pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry. J Clin Oncol 2006;24:4492-4498.
126.
MacSweeney F, Papagiannopoulos K, Goldstraw P, Sheppard MN, Corrin B, Nicholson AG: An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation. Am J Surg Pathol 2003;27:1139-1146.
127.
Lantuejoul S, Nicholson AG, Sartori G, Piolat C, Danel C, Brabencova E, et al: Mucinous cells in type 1 pulmonary congenital cystic adenomatoid malformation as mucinous bronchioloalveolar carcinoma precursors. Am J Surg Pathol 2007;31:961-969.
128.
Ioachimescu OC, Mehta AC: From cystic pulmonary airway malformation, to bronchioloalveolar carcinoma and adenocarcinoma of the lung. Eur Respir J 2005;26:1181-1187.
129.
Stacher E, Ullmann R, Halbwedl I, Gogg-Kammerer M, Boccon-Gibod L, Nicholson AG, et al: Atypical goblet cell hyperplasia in congenital cystic adenomatoid malformation as a possible preneoplasia for pulmonary adenocarcinoma in childhood: a genetic analysis. Hum Pathol 2004;35:565-570.
130.
Mani H, Shilo K, Galvin JR, Stocker JT, Franks TJ: Spectrum of precursor and invasive neoplastic lesions in type 1 congenital pulmonary airway malformation: case report and review of the literature. Histopathology 2007;51:561-565.
131.
Summers RJ, Shehata BM, Bleacher JC, Stockwell C, Rapkin L: Mucinous adenocarcinoma of the lung in association with congenital pulmonary airway malformation. J Pediatr Surg 2010;45:2256-2259.
132.
Hamvas A: Current technology in the diagnosis of developmentally related lung disorders. Neonatology 2012;101:353-359.
133.
Mimmi MC, Ballico M, Nakib G, Calcaterra V, Peiro JL, Marotta M, et al: Altered metabolic profile in congenital lung lesions revealed by 1H nuclear magnetic resonance spectroscopy. ISRN Anal Chem 2014;2014:391836.
134.
Fanos V, van den Anker J, Noto A, Mussap M, Atzori L: Metabolomics in neonatology: fact or fiction? Semin Fetal Neonatal Med 2013;18:3-12.
135.
Fanos V, Iacovidou N, Puddu M, Ottonello G, Noto A, Atzori L: Metabolomics in neonatal life. Early Hum Dev 2013;89:S7-S10.
136.
Pelizzo G, Mimmi MC, Ballico M, Marotta M, Goruppi I, Peiro JL, et al: Congenital pulmonary malformations: metabolomic profile of lung phenotype in infants. J Matern Fetal Neonatal Med 2016;29:143-147.
137.
Lezmi G, Verkarre V, Khen-Dunlop N, Vibhushan S, Hadchouel A, Rambaud C, et al: FGF10 Signaling differences between type I pleuropulmonary blastoma and congenital cystic adenomatoid malformation. Orphanet J Rare Dis 2013;8:130.
138.
Yin Y, Castro AM, Hoekstra M, Yan TJ, Kanakamedala AC, Dehner LP, et al: Fibroblast growth factor 9 regulation by microRNAs controls lung development and links DICER1 loss to the pathogenesis of pleuropulmonary blastoma. PLoS Genet 2015;11:e1005242.
139.
Esteban E, Majem M, Aguillo MM, Banaclocha NM, Dómine M, Aldaravi LG, et al: Prevalence of EGFR mutations in newly diagnosed locally advanced or metastatic non-small cell lung cancer Spanish patients and its association with histological subtypes and clinical features: the Spanish REASON study. Cancer Epidemiol 2015;39:291-297.
140.
Cass DL, Quinn TM, Yang EY, Liechty KW, Crombleholme TM, Flake AW, et al: Increased cell proliferation and decreased apoptosis characterize congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 1998;33:1043-1047.
141.
Hwang J, Kang MH, Yoo YA, Quan YH, Kim HK, Oh SC, et al: The effects of sonic hedgehog signaling pathway components on non-small-cell lung cancer progression and clinical outcome. World J Surg Oncol 2014;12:268.
142.
Sousa V, Bastos B, Silva M, Alarcao AM, Carvalho L: Bronchial-pulmonary adenocarcinoma subtyping relates with different molecular pathways. Rev Port Pneumol 2015;21:259-270.
143.
Yin Y, Betsuyaku T, Garbow JR, Miao J, Govindan R, Ornitz DM: Rapid induction of lung adenocarcinoma by fibroblast growth factor 9 signaling through FGF receptor 3. Cancer Res 2013;73:5730-5741.
144.
Parikh DH, Rasiah SV: Congenital lung lesions: postnatal management and outcome. Semin Pediatr Surg 2015;24:160-167.
145.
West D, Nicholson AG, Colquhoun I, Pollock J: Bronchioloalveolar carcinoma in congenital cystic adenomatoid malformation of lung. Ann Thorac Surg 2007;83:687-689.
146.
Endo T, Inoue M, Watanabe N, Tanaka I, Futonaka H, Takemura T: Two operative cases of pulmonary blastoma (in Japanese). Kyobu Geka 1982;35:219-224.
147.
Stephanopoulos C, Catsaras H: Myxosarcoma complicating a cystic hamartoma of the lung. Thorax 1963;18:144-145.
148.
Pai S, Eng HL, Lee SY, Hsiao CC, Huang WT, Huang SC: Rhabdomyosarcoma arising within congenital cystic adenomatoid malformation. Pediatr Blood Cancer 2005;45:841-845.
149.
Doladzas T, Arvelakis A, Karavokyros IG, Gougoudi E, Pikoulis E, Patsouris E, et al: Primary rhabdomyosarcoma of the lung arising over cystic pulmonary adenomatoid malformation. Pediatr Hematol Oncol 2005;22:525-529.
150.
Nur S, Badr R, Sandoval C, Brudniki A, Yeh A: Syndromic presentation of a pleuropulmonary blastoma associated with congenital cystic adenomatoid malformation: a case report. J Pediatr Surg 2007;42:1772-1775.
151.
Gutweiler JR, Labelle J, Suh MY, Misra MV, Ahmed M, Lee EY, et al: A familial case of pleuropulmonary blastoma. Eur J Pediatr Surgery 2008;18:192-194.
152.
Ishida M, Igarashi T, Teramoto K, Hanaoka J, Iwai M, Yoshida K, et al: Mucinous bronchioloalveolar carcinoma with K-ras mutation arising in type 1 congenital cystic adenomatoid malformation: a case report with review of the literature. Int J Clin Exp Pathol 2013;6:2597-2602.
153.
Kim MY, Kang CH, Park SH: Multifocal synchronous mucinous adenocarcinomas arising in congenital pulmonary airway malformation: a case report with molecular study. Histopathology 2014;65:926-932.
154.
Li J, Chen GS, Zhang X, Moore L, Cheng H: Congenital cystic adenomatoid malformation with associated mucinous bronchioloalveolar carcinoma in a neonate. Fetal Pediatr Pathol 2014;33:29-34.
155.
Abecasis F, Gomes Ferreira M, Oliveira A, Vaz Velho H: Bronchioloalveolar carcinoma associated with congenital pulmonary airway malformation in an asymptomatic adolescent (in Portuguese). Rev Port Pneumol 2008;14:285-290.
© 2016 S. Karger AG, Basel
2016
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.