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Congenital pulmonary airway malformation type 1

MedGen UID:
1760964
•Concept ID:
C5437763
•
Congenital Abnormality
Synonyms: CCAM type 1; Congenital cystic adenomatoid malformation of the lung type 1; congenital cystic adenomatoid malformation of the lung type 1; Congenital cystic adenomatous malformation of the lung type 1; congenital cystic adenomatous malformation of the lung type 1; Congenital cystic disease of the lung type 1; congenital cystic disease of the lung type 1; congenital pulmonary airway malformation type 1; CPAM type 1; Type 1 congenital pulmonary airway malformation
SNOMED CT: Congenital pulmonary airway malformation type 1 (890392004)
 
HPO: HP:0025725
Monarch Initiative: MONDO:0017249
Orphanet: ORPHA280832

Definition

A type of congenital pulmonary airway malformation that is characterized by multiple large cysts or a single dominant cyst. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital pulmonary airway malformation type 1

Professional guidelines

PubMed

Multimodality Imaging of Pleuropulmonary Blastoma: Pearls, Pitfalls, and Differential Diagnosis.
Leung SS, Donuru A, Kandula V, Parekh MR, Saul D
Semin Ultrasound CT MR 2022 Feb;43(1):61-72. Epub 2021 May 15 doi: 10.1053/j.sult.2021.05.007. PMID: 35164911
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
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
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
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
Fetal ultrasound and magnetic resonance imaging: a primer on how to interpret prenatal lung lesions.
Adams NC, Victoria T, Oliver ER, Moldenhauer JS, Adzick NS, Colleran GC
Pediatr Radiol 2020 Dec;50(13):1839-1854. Epub 2020 Nov 30 doi: 10.1007/s00247-020-04806-x. PMID: 33252753
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

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
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
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
Evidence to inform decisions about maternal-fetal surgery: technical brief.
Hartmann KE, McPheeters ML, Chescheir NC, Gillam-Krakauer M, McKoy JN, Jerome R, Sathe NA, Meints L, Walsh WF
Obstet Gynecol 2011 May;117(5):1191-1204. doi: 10.1097/AOG.0b013e318216151d. PMID: 21471854
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
Manifestation of congenital pulmonary airway malformation in a 26-year-old adult, requiring surgery.
Isowa M, Ueda S, Sonobe M, Date H
Interact Cardiovasc Thorac Surg 2019 Jul 1;29(1):154-156. doi: 10.1093/icvts/ivz032. PMID: 30844060
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

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
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: 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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