Congenital Pulmonary Malformations: Classification and Pathogenesis
Main Article Content
Abstract
Background: Congenital pulmonary malformations (CPMs) are rare developmental anomalies affecting the airways, lung parenchima, and intrathoracic vasculature. Their reported incidence has increased with the widespread use of prenatal imaging. Although often asymptomatic, some CPMs carry a risk of respiratory complications and malignant transformation.
Objective: To summarize current knowledge of the classification, pathogenesis, diagnosis imaging, and clinical implications of CPMs
Summary: CPMs result from disruptions in embryonic lung development and involve key signaling pathways such as SHH, WNT, BMP, FGF, and TGF-B. Mutations and transcription factors like NKX2.1 and SOX2, along with genetic alterations (e.g. KRAS, DICER) have been identified in several CPMs. Recent advances fetal images studies and postnatal CT angiography have improved early diagnosis and lesion characterization. Long-term studies reveal that even asymptomatic lesions may progress, with a subset showing malignant transformation.
Conclusion: A multidisciplinary approach is essential for managing CPMs. Understanding their molecular basis and identifying prognostic markers are critical for risk stratification and guiding treatment. Standarized classification and longitudinal studies are needed to optimize outcomes.
Keywords:
Congenital pulmonary malformations, CPM, lung development, prenatal imaging, malignancy risk
Article Details
How to Cite
SAAVEDRA B., Mónica; ORTEGA F., Ximena.
Congenital Pulmonary Malformations: Classification and Pathogenesis.
Medical Research Archives, [S.l.], v. 13, n. 7, july 2025.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/6775>. Date accessed: 06 dec. 2025.
doi: https://doi.org/10.18103/mra.v13i7.6775.
Section
Research Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
1. Dolk H. EUROCAT: 25 years of European surveillance of congenital anomalies. Arch Dis Child Fetal Neonatal Ed. 2005 Sep;90(5):F355–F358. doi:10.1136/adc.2004.062810
2. Lau CT, Kan A, Shek N, Tam P, Wong KKY. Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program. Pediatr Surg Int. 2017;33(1):105–108. doi:10.1007/s00383-016-3991-1
3. Wan H, Dingle S, Xu Y, Besnard V, Kaestner KH, Ang SL, et al. Compensatory roles of Foxa1 and Foxa2 during lung morphogenesis. J Biol Chem. 2005;280(14):13809–13816.
4. White AC, Xu J, Yin Y, Smith C, Schmid G, Ornitz DM. FGF9 and SHH signaling coordinate lung growth and development through regulation of distinct mesenchymal domains. Development. 2006 Apr;133(8):1507–1517. doi:10.1242/dev.02313. PMID: 16540513
5. Kuo CT, Morrisey EE, Anandappa R, Sigrist K, Lu MM, Parmacek MS, Soudais C, Leiden JM. GATA4 transcription factor is required for ventral morphogenesis and heart tube formation. Genes Dev. 1997 Apr 15;11(8):1048–1060. doi:10.1101/gad.11.8.1048. PMID: 9136932
6. Hrycaj SM, Dye BR, Baker NC, Larsen BM, Burke AC, Spence JR, et al. Hox5 genes regulate the Wnt2/2b-Bmp4-signaling axis during lung development. Cell Rep. 2015;12(6):903-912.
7. Domyan ET, Ferretti E, Throckmorton K, Mishina Y, Nicolis SK, Sun X. Signaling through BMP receptors promotes respiratory identity in the foregut via repression of Sox2. Development. 2011;138(5):971-981.
8. 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(1):296-309.
9. Kuwahara A, Lewis AE, Coombes C, Leung FS, Percharde M, Bush JO. Delineating the early transcriptional specification of the mammalian trachea and esophagus. Elife. 2020;9:e55526.
10. Volpe MV, Ramadurai SM, Mujahid S, Vong T, Brandao M, Wang KT, et al. Regulatory interactions between androgens, Hoxb5, and TGF-β signaling in murine lung development. Biomed Res Int. 2013;2013:320249.
11. Miao Q, Chen H, Luo Y, Chiu J, Chu L, Thornton ME, et al. Abrogation of mesenchyme-specific TGF-β signaling results in lung malformation with prenatal pulmonary cysts in mice. Am J Physiol Lung Cell Mol Physiol. 2021 Jun 1;320(6):L1158–L1168.
12. Volpe MAV, Pham L, Lessin M, Ralston SJ, Bhan I, Cutz E, et al. Expression of Hoxb5 during human lung development and in congenital lung malformations. Birth Defects Res A Clin Mol Teratol. 2003 Oct;67(8):550–556.
13. Lange AW, Sridharan A, Xu Y, Stripp BR, Perl AK, Whitsett JA. Hippo/Yap signaling controls epithelial progenitor cell proliferation and differentiation in the embryonic and adult lung. J Mol Cell Biol. 2015 Feb;7(1):35–47.
14. Parlak A, Ak Aksoy S, Erçelik M, Tekin Ç, Nazlıoğlu HÖ, Tunca B, Gürpınar AN, et al. Matrix metalloproteinase-7 and matrix metalloproteinase-9 expression is upregulated in congenital lung malformations. Turk J Pediatr. 2025 Feb 20;67(1):31–38.
15. Luo Y, Cao K, Chiu J, Chen H, Wang HJ, Thornton ME, et al. Defective mesenchymal Bmpr1a-mediated BMP signaling causes congenital pulmonary cysts. eLife. 2023 Feb 28;12:e83327. doi:10.7554/eLife.83327. PMID: 36864912
16. Zhang G, Lou L, Shen L, Zeng H, Cai C, Wu R, et al. The underlying molecular mechanism of ciliated epithelium dysfunction and TGF-β signaling in children with congenital pulmonary airway malformations. Sci Rep. 2024 Feb 28;14(1):4430.
17. 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 Mar;39(3):346–355.
18. Clark JC, Tichelaar JW, Wert SE, Itoh N, Perl AKT, Stahlman MT, et al. FGF 10 disrupts lung morphogenesis and causes pulmonary adenomas in vivo. Am J Physiol Lung Cell Mol Physiol. 2001 Apr;280(4):L705–L715.
19. 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 U S A. 1995 Feb 28;92(5):1851–1855.
20. 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 Sep-Oct;3(5):455–461. doi:10.1007/s100249900046. PMID: 11143507
21. Taylor B, Rice A, Nicholson AG, Hind M, Dean CH. Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations. Thorax. 2020 Nov;75(11):1001–1003. doi:10.1136/thoraxjnl-2020-214752.
22. Rockich BE, Hrycaj SM, Shih HP, Nagy MS, Ferguson MAH, Kopp JL, et al. Sox9 plays multiple roles in the lung epithelium during branching morphogenesis. Proc Natl Acad Sci U S A. 2013 Nov 19;110(47):E4456–E4464.
23. Salgia R, Pharaon R, Mambetsariev I, Nam A, Sattler M. The improbable targeted therapy: KRAS as an emerging target in non-small cell lung cancer (NSCLC). Cell Rep Med. 2021 Jan 19;2(1):100186.
24. Hermelijn SM, Wolf JL, den Toom TD, Wijnen RMH, Rottier RJ, Schnater JM, et al. Early KRAS oncogenic driver mutations in nonmucinous tissue of congenital pulmonary airway malformations. Hum Pathol. 2020 Sep;103:95–106.
25. Harris KS, Zhang Z, McManus MT, Harfe BD, Sun X. Dicer function is essential for lung epithelium morphogenesis. Proc Natl Acad Sci U S A. 2006 Feb 14;103(7):2208–2213.
26. Brcic L, Fakler F, Eidenhammer S, Thueringer A, Kashofer K, Kulka J, et al. Pleuropulmonary blastoma type I might arise in congenital pulmonary airway malformation type 4 by acquiring a Dicer1 mutation. Virchows Arch. 2020 Mar;477(3):375–382.
27. Zhang S, Ye C, Xiao J, Yang J, Zhu C, Xiao Y, et al. Single-cell transcriptome profiling reveals the mechanism of abnormal proliferation of epithelial cells in congenital cystic adenomatoid malformation. Exp Cell Res. 2020 Sep 1;396(2):112205.
28. Jeon HS, Dracheva T, Yang SH, Meerzaman D, Fukuoka J, Shakoori A, et al. SMAD6 contributes to patient survival in non-small cell lung cancer and its knockdown reestablishes TGF-β homeostasis in lung cancer cells. Cancer Res. 2008 Dec 1;68(23):9686–9692.
29. van Horik C, Zuidweg MJP, Boerema De Munck A, Buscop Van Kempen M, Brosens E, Vahrmeijer AL, et al. Selection of potential targets for stratifying congenital pulmonary airway malformation patients with molecular imaging: is MUC1 the one? Eur Respir Rev. 2023 Apr 26;32(177):230062.
30. Patrizi S, Pederiva F, d’Adamo AP. Whole genome methylation study of congenital lung malformations in children. Front Oncol. 2021 Nov 24;11:751925.
31. Rodrigues de Moura R, Patrizi S, Athanasakis E, Schleef J, Pederiva F, d’Adamo AP. Genomic instability in congenital lung malformations in children. Pediatr Surg Int. 2024 Jan;40(1):3–12.
32. Rankin SA, Han L, McCracken KW, Kenny AP, Anglin CT, Grigg EA, et al. A retinoic acid–Hedgehog cascade coordinates mesoderm inducing signals and endoderm competence during lung specification. Cell Rep. 2016 Jan 12;16(1):66–78.
33. Fernandes Silva H, Vaz Cunha P, Barbosa VB, Silva Gonçalves C, Correia Pinto J, Moura RS. Retinoic acid regulates avian lung branching through a molecular network. Cell Mol Life Sci. 2017 Dec;74(24):4599–4619.
34. Zhang G, Cai C, Li X, Lou L, Zhou B, Zeng H, et al. Application of second generation sequencing in congenital pulmonary airway malformations. Sci Rep. 2022 Feb 17;12(1):2986.
35. Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C, et al.; ERS Lung Congenital Malformation Task Force. Antenatal and postnatal management of congenital cystic adenomatoid malformation. Paediatr Respir Rev. 2012 Jun;13(2):77–86.
36. Bush A, Hogg J, Chitty LS. Cystic lung lesions: prenatal diagnosis and management. Prenat Diagn. 2008 May;28(7):604–611.
37. Cavoretto P, Molina F, Poggi S, Davenport M, Nicolaides KH. Prenatal diagnosis and outcome of echogenic fetal lung lesions. Ultrasound Obstet Gynecol. 2008 Dec;32(6):769–783.
38. Elders BBLJ, Kersten CM, Hermelijn SM, Wielopolski PA, Tiddens HAWM, Schnater JM, et al. Congenital lung abnormalities on magnetic resonance imaging: the CLAM study. Eur Radiol. 2023 Jul;33(7):4767–4779.
39. Pacharn P, Kline Fath B, Calvo Garcia M, Linam LE, Rubio EI, Salisbury S, et al. Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol. 2013 Sep;43(9):1136–1143.
40. Hermelijn SM, Dragt OV, Bosch JJ, Hijkoop A, Riera L, Ciet P, et al. Congenital lung abnormality quantification by computed tomography: the CLAQ method. Pediatr Pulmonol. 2020 Nov;55(11):3152–3161.
41. Langston C. New concepts in the pathology of congenital lung malformations. Semin Pediatr Surg. 2003 Feb;12(1):17–37.
42. Stocher JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung: classification and morphologic spectrum. Radiographics. 1994 Nov;14(6):1317–1327.
43. Yang W, Gao Y, Li P, Eckman MH. Should asymptomatic patients with congenital lung malformations undergo surgery? A decision analysis. Pediatr Pulmonol. 2023 Feb;58(2):449–456.
44. Pederiva F, Dalena P, Pasqua N, Bresesti I, Testa V, Zirpoli S, et al.; European Congenital Lung Malformation Working Group. Risk of malignant transformation and infections in congenital lung malformations in adults: a systematic review. Eur Respir Rev. 2025 Jan 15;34(183):240014.
45. Aziz D, Langer JC, Tuuha SE, Ryan G, Ein SH, Kim PCW, et al. Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not? J Pediatr Surg. 2004 Mar;39(3):329–334.
46. Nasr A, Himidan S, Pastor AC, Taylor G, Kim PCW. Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma? J Pediatr Surg. 2010 Jun;45(6):1086–1089.
2. Lau CT, Kan A, Shek N, Tam P, Wong KKY. Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program. Pediatr Surg Int. 2017;33(1):105–108. doi:10.1007/s00383-016-3991-1
3. Wan H, Dingle S, Xu Y, Besnard V, Kaestner KH, Ang SL, et al. Compensatory roles of Foxa1 and Foxa2 during lung morphogenesis. J Biol Chem. 2005;280(14):13809–13816.
4. White AC, Xu J, Yin Y, Smith C, Schmid G, Ornitz DM. FGF9 and SHH signaling coordinate lung growth and development through regulation of distinct mesenchymal domains. Development. 2006 Apr;133(8):1507–1517. doi:10.1242/dev.02313. PMID: 16540513
5. Kuo CT, Morrisey EE, Anandappa R, Sigrist K, Lu MM, Parmacek MS, Soudais C, Leiden JM. GATA4 transcription factor is required for ventral morphogenesis and heart tube formation. Genes Dev. 1997 Apr 15;11(8):1048–1060. doi:10.1101/gad.11.8.1048. PMID: 9136932
6. Hrycaj SM, Dye BR, Baker NC, Larsen BM, Burke AC, Spence JR, et al. Hox5 genes regulate the Wnt2/2b-Bmp4-signaling axis during lung development. Cell Rep. 2015;12(6):903-912.
7. Domyan ET, Ferretti E, Throckmorton K, Mishina Y, Nicolis SK, Sun X. Signaling through BMP receptors promotes respiratory identity in the foregut via repression of Sox2. Development. 2011;138(5):971-981.
8. 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(1):296-309.
9. Kuwahara A, Lewis AE, Coombes C, Leung FS, Percharde M, Bush JO. Delineating the early transcriptional specification of the mammalian trachea and esophagus. Elife. 2020;9:e55526.
10. Volpe MV, Ramadurai SM, Mujahid S, Vong T, Brandao M, Wang KT, et al. Regulatory interactions between androgens, Hoxb5, and TGF-β signaling in murine lung development. Biomed Res Int. 2013;2013:320249.
11. Miao Q, Chen H, Luo Y, Chiu J, Chu L, Thornton ME, et al. Abrogation of mesenchyme-specific TGF-β signaling results in lung malformation with prenatal pulmonary cysts in mice. Am J Physiol Lung Cell Mol Physiol. 2021 Jun 1;320(6):L1158–L1168.
12. Volpe MAV, Pham L, Lessin M, Ralston SJ, Bhan I, Cutz E, et al. Expression of Hoxb5 during human lung development and in congenital lung malformations. Birth Defects Res A Clin Mol Teratol. 2003 Oct;67(8):550–556.
13. Lange AW, Sridharan A, Xu Y, Stripp BR, Perl AK, Whitsett JA. Hippo/Yap signaling controls epithelial progenitor cell proliferation and differentiation in the embryonic and adult lung. J Mol Cell Biol. 2015 Feb;7(1):35–47.
14. Parlak A, Ak Aksoy S, Erçelik M, Tekin Ç, Nazlıoğlu HÖ, Tunca B, Gürpınar AN, et al. Matrix metalloproteinase-7 and matrix metalloproteinase-9 expression is upregulated in congenital lung malformations. Turk J Pediatr. 2025 Feb 20;67(1):31–38.
15. Luo Y, Cao K, Chiu J, Chen H, Wang HJ, Thornton ME, et al. Defective mesenchymal Bmpr1a-mediated BMP signaling causes congenital pulmonary cysts. eLife. 2023 Feb 28;12:e83327. doi:10.7554/eLife.83327. PMID: 36864912
16. Zhang G, Lou L, Shen L, Zeng H, Cai C, Wu R, et al. The underlying molecular mechanism of ciliated epithelium dysfunction and TGF-β signaling in children with congenital pulmonary airway malformations. Sci Rep. 2024 Feb 28;14(1):4430.
17. 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 Mar;39(3):346–355.
18. Clark JC, Tichelaar JW, Wert SE, Itoh N, Perl AKT, Stahlman MT, et al. FGF 10 disrupts lung morphogenesis and causes pulmonary adenomas in vivo. Am J Physiol Lung Cell Mol Physiol. 2001 Apr;280(4):L705–L715.
19. 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 U S A. 1995 Feb 28;92(5):1851–1855.
20. 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 Sep-Oct;3(5):455–461. doi:10.1007/s100249900046. PMID: 11143507
21. Taylor B, Rice A, Nicholson AG, Hind M, Dean CH. Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations. Thorax. 2020 Nov;75(11):1001–1003. doi:10.1136/thoraxjnl-2020-214752.
22. Rockich BE, Hrycaj SM, Shih HP, Nagy MS, Ferguson MAH, Kopp JL, et al. Sox9 plays multiple roles in the lung epithelium during branching morphogenesis. Proc Natl Acad Sci U S A. 2013 Nov 19;110(47):E4456–E4464.
23. Salgia R, Pharaon R, Mambetsariev I, Nam A, Sattler M. The improbable targeted therapy: KRAS as an emerging target in non-small cell lung cancer (NSCLC). Cell Rep Med. 2021 Jan 19;2(1):100186.
24. Hermelijn SM, Wolf JL, den Toom TD, Wijnen RMH, Rottier RJ, Schnater JM, et al. Early KRAS oncogenic driver mutations in nonmucinous tissue of congenital pulmonary airway malformations. Hum Pathol. 2020 Sep;103:95–106.
25. Harris KS, Zhang Z, McManus MT, Harfe BD, Sun X. Dicer function is essential for lung epithelium morphogenesis. Proc Natl Acad Sci U S A. 2006 Feb 14;103(7):2208–2213.
26. Brcic L, Fakler F, Eidenhammer S, Thueringer A, Kashofer K, Kulka J, et al. Pleuropulmonary blastoma type I might arise in congenital pulmonary airway malformation type 4 by acquiring a Dicer1 mutation. Virchows Arch. 2020 Mar;477(3):375–382.
27. Zhang S, Ye C, Xiao J, Yang J, Zhu C, Xiao Y, et al. Single-cell transcriptome profiling reveals the mechanism of abnormal proliferation of epithelial cells in congenital cystic adenomatoid malformation. Exp Cell Res. 2020 Sep 1;396(2):112205.
28. Jeon HS, Dracheva T, Yang SH, Meerzaman D, Fukuoka J, Shakoori A, et al. SMAD6 contributes to patient survival in non-small cell lung cancer and its knockdown reestablishes TGF-β homeostasis in lung cancer cells. Cancer Res. 2008 Dec 1;68(23):9686–9692.
29. van Horik C, Zuidweg MJP, Boerema De Munck A, Buscop Van Kempen M, Brosens E, Vahrmeijer AL, et al. Selection of potential targets for stratifying congenital pulmonary airway malformation patients with molecular imaging: is MUC1 the one? Eur Respir Rev. 2023 Apr 26;32(177):230062.
30. Patrizi S, Pederiva F, d’Adamo AP. Whole genome methylation study of congenital lung malformations in children. Front Oncol. 2021 Nov 24;11:751925.
31. Rodrigues de Moura R, Patrizi S, Athanasakis E, Schleef J, Pederiva F, d’Adamo AP. Genomic instability in congenital lung malformations in children. Pediatr Surg Int. 2024 Jan;40(1):3–12.
32. Rankin SA, Han L, McCracken KW, Kenny AP, Anglin CT, Grigg EA, et al. A retinoic acid–Hedgehog cascade coordinates mesoderm inducing signals and endoderm competence during lung specification. Cell Rep. 2016 Jan 12;16(1):66–78.
33. Fernandes Silva H, Vaz Cunha P, Barbosa VB, Silva Gonçalves C, Correia Pinto J, Moura RS. Retinoic acid regulates avian lung branching through a molecular network. Cell Mol Life Sci. 2017 Dec;74(24):4599–4619.
34. Zhang G, Cai C, Li X, Lou L, Zhou B, Zeng H, et al. Application of second generation sequencing in congenital pulmonary airway malformations. Sci Rep. 2022 Feb 17;12(1):2986.
35. Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C, et al.; ERS Lung Congenital Malformation Task Force. Antenatal and postnatal management of congenital cystic adenomatoid malformation. Paediatr Respir Rev. 2012 Jun;13(2):77–86.
36. Bush A, Hogg J, Chitty LS. Cystic lung lesions: prenatal diagnosis and management. Prenat Diagn. 2008 May;28(7):604–611.
37. Cavoretto P, Molina F, Poggi S, Davenport M, Nicolaides KH. Prenatal diagnosis and outcome of echogenic fetal lung lesions. Ultrasound Obstet Gynecol. 2008 Dec;32(6):769–783.
38. Elders BBLJ, Kersten CM, Hermelijn SM, Wielopolski PA, Tiddens HAWM, Schnater JM, et al. Congenital lung abnormalities on magnetic resonance imaging: the CLAM study. Eur Radiol. 2023 Jul;33(7):4767–4779.
39. Pacharn P, Kline Fath B, Calvo Garcia M, Linam LE, Rubio EI, Salisbury S, et al. Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol. 2013 Sep;43(9):1136–1143.
40. Hermelijn SM, Dragt OV, Bosch JJ, Hijkoop A, Riera L, Ciet P, et al. Congenital lung abnormality quantification by computed tomography: the CLAQ method. Pediatr Pulmonol. 2020 Nov;55(11):3152–3161.
41. Langston C. New concepts in the pathology of congenital lung malformations. Semin Pediatr Surg. 2003 Feb;12(1):17–37.
42. Stocher JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung: classification and morphologic spectrum. Radiographics. 1994 Nov;14(6):1317–1327.
43. Yang W, Gao Y, Li P, Eckman MH. Should asymptomatic patients with congenital lung malformations undergo surgery? A decision analysis. Pediatr Pulmonol. 2023 Feb;58(2):449–456.
44. Pederiva F, Dalena P, Pasqua N, Bresesti I, Testa V, Zirpoli S, et al.; European Congenital Lung Malformation Working Group. Risk of malignant transformation and infections in congenital lung malformations in adults: a systematic review. Eur Respir Rev. 2025 Jan 15;34(183):240014.
45. Aziz D, Langer JC, Tuuha SE, Ryan G, Ein SH, Kim PCW, et al. Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not? J Pediatr Surg. 2004 Mar;39(3):329–334.
46. Nasr A, Himidan S, Pastor AC, Taylor G, Kim PCW. Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma? J Pediatr Surg. 2010 Jun;45(6):1086–1089.