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Congenital pulmonary airway malformation type 4

MedGen UID:
1777484
•Concept ID:
C5437760
•
Congenital Abnormality
Synonyms: Congenital cystic adenomatoid malformation of the lung type 4; congenital cystic adenomatoid malformation of the lung type 4; congenital cystic adenomatous malformation of the lung type 4; Congenital cystic adenomatous malformation of the lung type 4; congenital pulmonary airway malformation type 4; CPAM type 4
SNOMED CT: Congenital pulmonary airway malformation type 4 (890388006)
 
Monarch Initiative: MONDO:0017252
Orphanet: ORPHA280854

Definition

Congenital pulmonary airway malformation type 4 originates in the acinar structures of the lung. It consists of peripheral thin-walled cysts, typically affecting a single lobe. There is a strong association with type 1 pleuropulmonary blastoma. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital pulmonary airway malformation type 4

Professional guidelines

PubMed

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases.
Giubergia V, Barrenechea M, Siminovich M, Pena HG, Murtagh P
J Pediatr (Rio J) 2012 Mar-Apr;88(2):143-8. doi: 10.2223/JPED.2177. PMID: 22543527
Cystic adenomatoid malformation of the lung: prenatal diagnosis, prognostic factors and fetal outcome.
Golaszewski T, Bettelheim D, Eppel W, Deutinger J, Bernaschek G
Gynecol Obstet Invest 1998;46(4):241-6. doi: 10.1159/000010042. PMID: 9813442
Prenatal management of congenital cystic adenomatoid malformation of the lung.
Dumez Y, Mandelbrot L, Radunovic N, Révillon Y, Dommergues M, Aubry MC, Aubry JP, Narcy F, Sonigo P
J Pediatr Surg 1993 Jan;28(1):36-41. doi: 10.1016/s0022-3468(05)80350-1. PMID: 8429468

Recent clinical studies

Etiology

Neonatal congenital pulmonary airway malformation associated with mucinous adenocarcinoma and KRAS mutations.
Muntean A, Banias LE, Ade-Ajayi N, Patel SB, McKinney O, Davenport M
J Pediatr Surg 2022 Nov;57(11):520-526. Epub 2021 Dec 25 doi: 10.1016/j.jpedsurg.2021.12.018. PMID: 34980466
An investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia.
Watanabe T, Ohno M, Tahara K, Tomonaga K, Fuchimoto Y, Fujino A, Hishiki T, Tsukamoto K, Ito Y, Sugibayashi R, Wada S, Sago H, Higuchi M, Kawasaki K, Yoshioka T, Kanamori Y
J Pediatr Surg 2018 Dec;53(12):2390-2393. Epub 2018 Sep 1 doi: 10.1016/j.jpedsurg.2018.08.031. PMID: 30227995
Congenital pulmonary airway malformations: state-of-the-art review for pediatrician's use.
Leblanc C, Baron M, Desselas E, Phan MH, Rybak A, Thouvenin G, Lauby C, Irtan S
Eur J Pediatr 2017 Dec;176(12):1559-1571. Epub 2017 Oct 19 doi: 10.1007/s00431-017-3032-7. PMID: 29046943
Association between Congenital Lung Malformations and Lung Tumors in Children and Adults: A Systematic Review.
Casagrande A, Pederiva F
J Thorac Oncol 2016 Nov;11(11):1837-1845. Epub 2016 Jul 15 doi: 10.1016/j.jtho.2016.06.023. PMID: 27423390
Congenital pulmonary airway malformation: CT-pathologic correlation.
Shimohira M, Hara M, Kitase M, Takeuchi M, Shibamoto Y, Kurono K, Shimizu S
J Thorac Imaging 2007 May;22(2):149-53. doi: 10.1097/01.rti.0000213586.06602.d3. PMID: 17527118

Diagnosis

Congenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification.
Dehner LP, Schultz KAP, Hill DA
Pediatr Dev Pathol 2023 May-Jun;26(3):241-249. Epub 2023 Feb 21 doi: 10.1177/10935266221146823. PMID: 37334833
Neonatal congenital pulmonary airway malformation associated with mucinous adenocarcinoma and KRAS mutations.
Muntean A, Banias LE, Ade-Ajayi N, Patel SB, McKinney O, Davenport M
J Pediatr Surg 2022 Nov;57(11):520-526. Epub 2021 Dec 25 doi: 10.1016/j.jpedsurg.2021.12.018. PMID: 34980466
Congenital pulmonary airway malformations: state-of-the-art review for pediatrician's use.
Leblanc C, Baron M, Desselas E, Phan MH, Rybak A, Thouvenin G, Lauby C, Irtan S
Eur J Pediatr 2017 Dec;176(12):1559-1571. Epub 2017 Oct 19 doi: 10.1007/s00431-017-3032-7. PMID: 29046943
Association between Congenital Lung Malformations and Lung Tumors in Children and Adults: A Systematic Review.
Casagrande A, Pederiva F
J Thorac Oncol 2016 Nov;11(11):1837-1845. Epub 2016 Jul 15 doi: 10.1016/j.jtho.2016.06.023. PMID: 27423390
Congenital pulmonary airway malformation: a case report and review of the literature.
McDonough RJ, Niven AS, Havenstrite KA
Respir Care 2012 Feb;57(2):302-6. Epub 2011 Jul 12 doi: 10.4187/respcare.00727. PMID: 21762550

Therapy

Early vs late resection of asymptomatic congenital lung malformations.
Style CC, Cass DL, Verla MA, Cruz SM, Lau PE, Lee TC, Fernandes CJ, Keswani SG, Olutoye OO
J Pediatr Surg 2019 Jan;54(1):70-74. Epub 2018 Oct 5 doi: 10.1016/j.jpedsurg.2018.10.035. PMID: 30366720
Outcome of infants operated on for congenital pulmonary malformations.
Calzolari F, Braguglia A, Valfrè L, Dotta A, Bagolan P, Morini F
Pediatr Pulmonol 2016 Dec;51(12):1367-1372. Epub 2016 May 27 doi: 10.1002/ppul.23472. PMID: 27232731
Nasoethmoidal meningocele in a child presenting bilateral congenital cystic adenomatoid malformation: Evidence for a new entity or consequence of gestational exposures?
da Rosa EB, Silveira DB, Tsugami LG, Bellé NL, Matos IO, Targa LV, Betat Rda S, da Cunha AC, Villacis RA, Rogatto SR, Dorfman LE, Rosa RF, Zen PR
Birth Defects Res A Clin Mol Teratol 2016 Apr;106(4):225-31. Epub 2016 Mar 2 doi: 10.1002/bdra.23452. PMID: 26932830
Treatment of congenital pulmonary airway malformation induced hydrops fetalis via percutaneous sclerotherapy.
Lee FL, Said N, Grikscheit TC, Shin CE, Llanes A, Chmait RH
Fetal Diagn Ther 2012;31(4):264-8. Epub 2012 Feb 18 doi: 10.1159/000336226. PMID: 22354268
Congenital pulmonary airway malformation: CT-pathologic correlation.
Shimohira M, Hara M, Kitase M, Takeuchi M, Shibamoto Y, Kurono K, Shimizu S
J Thorac Imaging 2007 May;22(2):149-53. doi: 10.1097/01.rti.0000213586.06602.d3. PMID: 17527118

Prognosis

Mucinous adenocarcinoma arising in congenital pulmonary airway malformation: clinicopathological analysis of 37 cases.
Chang WC, Zhang YZ, Wolf JL, Hermelijn SM, Schnater JM, von der Thüsen JH, Rice A, Lantuejoul S, Mastroianni B, Farver C, Black F, Popat S, Nicholson AG
Histopathology 2021 Feb;78(3):434-444. Epub 2020 Oct 23 doi: 10.1111/his.14239. PMID: 32810914
Congenital Cystic Adenomatoid Malformation Volume Ratio in Prenatal Assessment of Prognosis of Fetal Pulmonary Sequestrations.
An P, Wang Y, Feng W, Zhang JQ, Ning YX, Yin JB, Ye HB, Song J, Chen XN, Xu JZ, He QY, Zeng H, Li Y, Yuan W, He ZZ
Curr Med Sci 2019 Aug;39(4):658-662. Epub 2019 Jul 25 doi: 10.1007/s11596-019-2088-1. PMID: 31347005
An investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia.
Watanabe T, Ohno M, Tahara K, Tomonaga K, Fuchimoto Y, Fujino A, Hishiki T, Tsukamoto K, Ito Y, Sugibayashi R, Wada S, Sago H, Higuchi M, Kawasaki K, Yoshioka T, Kanamori Y
J Pediatr Surg 2018 Dec;53(12):2390-2393. Epub 2018 Sep 1 doi: 10.1016/j.jpedsurg.2018.08.031. PMID: 30227995
Congenital pulmonary airway malformation: CT-pathologic correlation.
Shimohira M, Hara M, Kitase M, Takeuchi M, Shibamoto Y, Kurono K, Shimizu S
J Thorac Imaging 2007 May;22(2):149-53. doi: 10.1097/01.rti.0000213586.06602.d3. PMID: 17527118
Congenital cystic adenomatoid malformation of the lung: prenatal management and prognosis.
Revillon Y, Jan D, Plattner V, Sonigo P, Dommergues M, Mandelbrot L, Dumez Y, Nihoul-Fékété C
J Pediatr Surg 1993 Aug;28(8):1009-11. doi: 10.1016/0022-3468(93)90504-e. PMID: 8229585

Clinical prediction guides

Congenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification.
Dehner LP, Schultz KAP, Hill DA
Pediatr Dev Pathol 2023 May-Jun;26(3):241-249. Epub 2023 Feb 21 doi: 10.1177/10935266221146823. PMID: 37334833
Mucinous adenocarcinoma arising in congenital pulmonary airway malformation: clinicopathological analysis of 37 cases.
Chang WC, Zhang YZ, Wolf JL, Hermelijn SM, Schnater JM, von der Thüsen JH, Rice A, Lantuejoul S, Mastroianni B, Farver C, Black F, Popat S, Nicholson AG
Histopathology 2021 Feb;78(3):434-444. Epub 2020 Oct 23 doi: 10.1111/his.14239. PMID: 32810914
Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features?
Feinberg A, Hall NJ, Williams GM, Schultz KA, Miniati D, Hill DA, Dehner LP, Messinger YH, Langer JC
J Pediatr Surg 2016 Jan;51(1):33-7. Epub 2015 Oct 23 doi: 10.1016/j.jpedsurg.2015.10.019. PMID: 26561249Free PMC Article
Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases.
Giubergia V, Barrenechea M, Siminovich M, Pena HG, Murtagh P
J Pediatr (Rio J) 2012 Mar-Apr;88(2):143-8. doi: 10.2223/JPED.2177. PMID: 22543527
Congenital pulmonary airway malformation: CT-pathologic correlation.
Shimohira M, Hara M, Kitase M, Takeuchi M, Shibamoto Y, Kurono K, Shimizu S
J Thorac Imaging 2007 May;22(2):149-53. doi: 10.1097/01.rti.0000213586.06602.d3. PMID: 17527118

Recent systematic reviews

Association between Congenital Lung Malformations and Lung Tumors in Children and Adults: A Systematic Review.
Casagrande A, Pederiva F
J Thorac Oncol 2016 Nov;11(11):1837-1845. Epub 2016 Jul 15 doi: 10.1016/j.jtho.2016.06.023. PMID: 27423390

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