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Congenital pulmonary airway malformation type 3

MedGen UID:
1741544
•Concept ID:
C5437762
•
Congenital Abnormality
Synonyms: CCAM type 3; Congenital cystic adenomatoid malformation of the lung type 3; congenital cystic adenomatoid malformation of the lung type 3; congenital cystic adenomatous malformation of the lung type 3; Congenital cystic adenomatous malformation of the lung type 3; Congenital cystic disease of the lung type 3; congenital cystic disease of the lung type 3; congenital pulmonary airway malformation type 3; CPAM type 3; Type 3 congenital pulmonary airway malformation
SNOMED CT: Congenital pulmonary airway malformation type 3 (890391006)
 
HPO: HP:0025727
Monarch Initiative: MONDO:0017251
Orphanet: ORPHA280847

Definition

A type of congenital pulmonary airway malformation that is characterized by a bulky firm mass with an adenomatoid appearance. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital pulmonary airway malformation type 3

Professional guidelines

PubMed

Obstetrician patterns of steroid administration for the prenatal management of congenital pulmonary airway malformations.
Aziz KB, Jelin AC, Keiser AM, Schulkin J, Jelin EB
J Neonatal Perinatal Med 2021;14(2):213-222. doi: 10.3233/NPM-200526. PMID: 33164951Free PMC Article
Prenatal imaging and postnatal presentation, diagnosis and management of congenital lung malformations.
Wall J, Coates A
Curr Opin Pediatr 2014 Jun;26(3):315-9. doi: 10.1097/MOP.0000000000000091. PMID: 24739492
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

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
Obstetric and neonatal outcomes in pregnancies complicated by fetal lung masses: does final histology matter?().
Anderson JN, Girsen AI, Hintz SR, El-Sayed YY, Davis AS, Barth RA, Halabi S, Hazard FK, Sylvester KG, Bruzoni M, Blumenfeld YJ
J Matern Fetal Neonatal Med 2021 Nov;34(22):3662-3668. Epub 2019 Nov 13 doi: 10.1080/14767058.2019.1689559. PMID: 31722592
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
Prenatal imaging and postnatal presentation, diagnosis and management of congenital lung malformations.
Wall J, Coates A
Curr Opin Pediatr 2014 Jun;26(3):315-9. doi: 10.1097/MOP.0000000000000091. PMID: 24739492
Congenital lung lesions--underlying molecular mechanisms.
Correia-Pinto J, Gonzaga S, Huang Y, Rottier R
Semin Pediatr Surg 2010 Aug;19(3):171-9. doi: 10.1053/j.sempedsurg.2010.03.003. PMID: 20610189

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
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
Prenatal imaging and postnatal presentation, diagnosis and management of congenital lung malformations.
Wall J, Coates A
Curr Opin Pediatr 2014 Jun;26(3):315-9. doi: 10.1097/MOP.0000000000000091. PMID: 24739492
Congenital lung lesions--underlying molecular mechanisms.
Correia-Pinto J, Gonzaga S, Huang Y, Rottier R
Semin Pediatr Surg 2010 Aug;19(3):171-9. doi: 10.1053/j.sempedsurg.2010.03.003. PMID: 20610189

Therapy

Obstetrician patterns of steroid administration for the prenatal management of congenital pulmonary airway malformations.
Aziz KB, Jelin AC, Keiser AM, Schulkin J, Jelin EB
J Neonatal Perinatal Med 2021;14(2):213-222. doi: 10.3233/NPM-200526. PMID: 33164951Free PMC Article
EGFR mutation of adenocarcinoma in congenital cystic adenomatoid malformation/congenital pulmonary airway malformation: a case report.
Hasegawa M, Sakai F, Arimura K, Katsura H, Koh E, Sekine Y, Hiroshima K
Jpn J Clin Oncol 2014 Mar;44(3):278-81. Epub 2014 Jan 30 doi: 10.1093/jjco/hyt226. PMID: 24482415
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
Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation, type 2: report of 50 cases.
Conran RM, Stocker JT
Pediatr Dev Pathol 1999 Sep-Oct;2(5):454-63. doi: 10.1007/s100249900149. PMID: 10441623

Prognosis

Multidisciplinary management of a large microcystic congenital pulmonary airway malformation: case report and literature review.
Chou JT, Kalantarova A, Borkowska-Kłos M, Kornacki J, Szczapa T, Wender-Ozegowska E
J Matern Fetal Neonatal Med 2023 Dec;36(2):2239424. doi: 10.1080/14767058.2023.2239424. PMID: 37581300
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
Prenatal imaging and postnatal presentation, diagnosis and management of congenital lung malformations.
Wall J, Coates A
Curr Opin Pediatr 2014 Jun;26(3):315-9. doi: 10.1097/MOP.0000000000000091. PMID: 24739492
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

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
Prediction of postnatal outcome in fetuses with congenital lung malformation: 2-year follow-up study.
Peters NCJ, Hijkoop A, Hermelijn SM, van Schoonhoven MM, Eggink AJ, van Rosmalen J, Otter SCMC, Tibboel D, IJsselstijn H, Schnater JM, Cohen-Overbeek TE
Ultrasound Obstet Gynecol 2021 Sep;58(3):428-438. doi: 10.1002/uog.23542. PMID: 33206446
Obstetrician patterns of steroid administration for the prenatal management of congenital pulmonary airway malformations.
Aziz KB, Jelin AC, Keiser AM, Schulkin J, Jelin EB
J Neonatal Perinatal Med 2021;14(2):213-222. doi: 10.3233/NPM-200526. PMID: 33164951Free PMC Article
Prenatal imaging and postnatal presentation, diagnosis and management of congenital lung malformations.
Wall J, Coates A
Curr Opin Pediatr 2014 Jun;26(3):315-9. doi: 10.1097/MOP.0000000000000091. PMID: 24739492
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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