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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

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
Prenatal course and postnatal management of peripheral bronchial atresia: association with congenital cystic adenomatoid malformation of the lung.
Peranteau WH, Merchant AM, Hedrick HL, Liechty KW, Howell LJ, Flake AW, Wilson RD, Johnson MP, Bebbington MW, Adzick NS
Fetal Diagn Ther 2008;24(3):190-6. Epub 2008 Aug 28 doi: 10.1159/000151337. PMID: 18753756
Percutaneous thoracic drainage in neonates: catheter drainage versus treatment with aspiration alone.
Margau R, Amaral JG, Chait PG, Cohen J
Radiology 2006 Oct;241(1):223-7. Epub 2006 Aug 23 doi: 10.1148/radiol.2411050966. PMID: 16928976

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
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
Thoracoamniotic Shunts in Macrocystic Lung Lesions: Case Series and Review of the Literature.
Litwińska M, Litwińska E, Janiak K, Piaseczna-Piotrowska A, Gulczyńska E, Szaflik K
Fetal Diagn Ther 2017;41(3):179-183. Epub 2016 Aug 5 doi: 10.1159/000448095. PMID: 27489957
Surgical management of bronchopulmonary malformations.
Wagenaar AE, Tashiro J, Hirzel A, Rodriguez LI, Perez EA, Hogan AR, Neville HL, Sola JE
J Surg Res 2015 Oct;198(2):406-12. Epub 2015 Apr 1 doi: 10.1016/j.jss.2015.03.080. PMID: 25918001
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

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 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
Surgical management of bronchopulmonary malformations.
Wagenaar AE, Tashiro J, Hirzel A, Rodriguez LI, Perez EA, Hogan AR, Neville HL, Sola JE
J Surg Res 2015 Oct;198(2):406-12. Epub 2015 Apr 1 doi: 10.1016/j.jss.2015.03.080. PMID: 25918001
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

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
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 cystic adenomatoid malformation of the lung: indications and timing of surgery.
Khosa JK, Leong SL, Borzi PA
Pediatr Surg Int 2004 Jul;20(7):505-8. Epub 2004 Jun 8 doi: 10.1007/s00383-004-1225-4. PMID: 15185110
Bronchioloalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation.
Granata C, Gambini C, Balducci T, Toma P, Michelazzi A, Conte M, Jasonni V
Pediatr Pulmonol 1998 Jan;25(1):62-6. doi: 10.1002/(sici)1099-0496(199801)25:1<62::aid-ppul8>3.0.co;2-q. PMID: 9475333

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
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 lung anomalies: can we postpone resection?
Colon N, Schlegel C, Pietsch J, Chung DH, Jackson GP
J Pediatr Surg 2012 Jan;47(1):87-92. doi: 10.1016/j.jpedsurg.2011.10.027. PMID: 22244398
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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