Congenital Cystic Adenomatoid Malformation of Lung in Adults_West China Medical Journal

Authors：

 LiuQian ¹ , LiDan ² , DingWuwu ¹ , ZouQing ³ ,  WuNing ¹ , MengHong ¹

1. Department of Pathology, the People’s Hospital of Deyang, Deyang, Sichuan 618000, P. R. China;
2. Department of Ultrasound, the Traditional Chinese Medicine Hospital of Leshan, Leshan, Sichuan 614000, P. R. China;
3. Department of Radiology, the People’s Hospital of Deyang, Deyang, Sichuan 618000, P. R. China;

Corresponding author：

WuNing, Email: wuning62@163.com

Keywords：

Lung; Congenital cystic adenomatoid malformation; Cyst; Clinicopathology

DOI：

10.7507/1002-0179.201600193

Video：

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Objective To investigate the clinicopathologic features of congenital cystic adenomatoid malformation (CCAM) of lung in adults. Methods The clinical and pathological characteristics of two cases of CCAM of lung in adults from November 19, 2012 to February 12, 2014 were analyzed, and relevant literatures were reviewed. Results Both of the two patients were males who were 59 years and 60 years old respectively. Both of them presented with respiratory symptoms such as productive cough, hemoptysis and fever. The lesions in the two cases were about 4.0 cm×3.0 cm×1.5 cm and 5.0 cm×5.0 cm×3.0 cm in size respectively. Both had a cystic appearance and involved unilateral lobes of the lung. Histologically, normal pulmonary alveoli were replaced by different size of cysts composed of adenomatoid hyperplastic bronchioles. The inner cystic wall was lined by pseudostratified ciliated columnar epithelium, and the cystic wall contained smooth muscle and elastic tissue, but no cartilage. In one of our cases, mucous cells could be seen in part of the inner cystic wall, with focal atypical hyperplasia. Conclusions CCAM of the lung is a rare congenital developmental anomaly, which typically manifests in neonates and infants, but extremely rare in adults. The diagnosis of CCAM in adults depends on clinical features, imaging changes and histopathological characteristics.

Citation： LiuQian, LiDan, DingWuwu, ZouQing, WuNing, MengHong. Congenital Cystic Adenomatoid Malformation of Lung in Adults. West China Medical Journal, 2016, 31(4): 710-713. doi: 10.7507/1002-0179.201600193 Copy

1.	Ch’In KY, Tang MY. Congenital adenomatoid malformation of one lobe of a lung with general anasarca[J]. Arch Pathol(Chic), 1949, 48(3): 221-229.
2.	Duncombe GJ, Dickinson JE, Kikiros CS. Prenatal diagnosis and management of congenital cystic adenomatoid malformation of the lung[J]. Am J Obstet Gynecol, 2002, 187(4): 950-954.
3.	Morelli L, Piscioli I, Licci S, et al. Pulmonary congenital cystic adenomatoid malformation, typeⅠ, presenting as a single cyst of the middle lobe in an adult: case report[J]. Diagn Pathol, 2007, 2: 17.
4.	Kim WS, Lee KS, Kim IO, et al. Congenital cystic adenomatoid malformation of the lung: CT-pathologic correlation[J]. AJR Am J Roentgenol, 1997, 168(1): 47-53.
5.	Shanmugam G. Adult congenital lung disease[J]. Eur J Cardiothorac Surg, 2005, 28(3): 483-489.
6.	Oh BJ, Lee JS, Kim JS, et al. Congenital cystic adenomatoid malformation of the lung in adults: clinical and CT evaluation of seven patients[J]. Respirology, 2006, 11(4): 496-501.
7.	Dahabreh J, Zisis C, Vassiliou M, et al. Congenital cystic adenomatoid malformation in an adult presenting as lung abscess[J]. Eur J Cardiothorac Surg, 2000, 18(6): 720-723.
8.	Belcher E, Lawson MH, Nicholson AG, et al. Congenital cystic adenomatoid malformation presenting as in-flight systemic air embolisation[J]. Eur Respir J, 2007, 30(4): 801-804.
9.	Kim SR, Lee MH, Lee KA, et al. A 38-year-old woman with bilateral cystic lesions in both lower lung lobes[J]. Chest, 2011, 140(2): 544-548.
10.	Feng A, Cai H, Sun Q, et al. Congenital cystic adenomatoid malformation of lung in adults: 2 rare cases report and review of the literature[J]. Diagn Pathol, 2012, 7: 37.
11.	Khan NU, Jones MT, Greaves M. Congenital cystic adenomatoid malformation of an entire lung in a 33-year-old man: a case report and review of the literature[J]. Br J Radiol, 2008, 81(971): 276-278.
12.	Cardoso WV, Itoh A, Nogawa H, et al. FGF-1 and FGF-7 induce distinct patterns of growth and differentiation in embryonic lung epithelium[J]. Dev Dyn, 1997, 208(3): 398-405.
13.	Cass DL, Quinn TM, Yang EY, et al. Increased cell proliferation and decreased apoptosis characterize congenital cystic adenomatoid malformation of the lung[J]. J Pediatr Surg, 1998, 33(7): 1043-1046.
14.	Cloutier MM, Schaeffer DA, Hight D. Congenital cystic adenomatoid malformation[J]. Chest, 1993, 103(3): 761-764.
15.	Mas A, Mirapeix RM, Domingo C, et al. Pulmonary hypoplasia presented in adulthood as a chronic respiratory failure: report of two cases. Embryology, clinical symptoms and diagnostic procedures[J]. Respiration, 1997, 64(3): 240-243.
16.	Roberts D, Sweeney E, Walkinshaw S. Congenital cystic adenomatoid malformation of the lung coexisting with recombinant chromosome 18. A case report[J]. Fetal diagn ther, 2001, 16(2): 65-67.
17.	Moerman P, Fryns JP, Vandenberghe K, et al. Pathogenesis of congenital cystic adenomatoid malformation of the lung[J]. Histopathology, 1992, 21(4): 315-321.
18.	Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum[J]. Hum Pathol, 1977, 8(2): 155-171.
19.	Stocker J. Congenital pulmonary airway malformation-a new name for and an expanded classification of congenital cystic adenomatoid malformation of the lung[J]. Histopathology, 2002, 41(Supp1): 424-431.
20.	Collins AM, Ridgway PF, Killeen RP, et al. Congenital cystic adenomatoid malformation of the lung: hazards of delayed diagnosis[J]. Respirology, 2009, 14(7): 1058-1060.
21.	常占平, 金锦善, 金妍, 等. 成人肺先天性囊性腺瘤样畸形伴发肺癌[J]. 诊断病理学杂志, 2003, 10(5): 284-286.
22.	MacSweeney F, Papagiannopoulos K, Goldstraw P, et al. An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation[J]. Am J Surg Pathol, 2003, 27(8): 1139-1146.
23.	Doladzas T, Arvelakis A, Karavokyros IG, et al. Primary rhabdomyosarcoma of the lung arising over cystic pulmonary adenomatoid malformation[J]. Pediatr Hematol Oncol, 2005, 22(6): 525-529.
24.	房惠琼, 李启明, 陶祥. 成人先天性肺囊性腺瘤样畸形伴类癌一例[J]. 中华病理学杂志, 2008, 37(10): 713-715.
25.	Stacher E, Ullmann R, Halbwedl I, et al. Atypical goblet cell hyperplasia in congenital cystic adenomatoid malformation as a possible preneoplasia for pulmonary adenocarcinoma in childhood: a genetic analysis[J]. Hum Pathol, 2004, 35(5): 565-570.
26.	Marshall KW, Blane CE, Teitelbaum DH, et al. Congenital cystic adenomatoid malformation: impact of prenatal diagnosis and changing strategies in the treatment of the asymptomatic patient[J]. AJR Am J Roentgenol, 2000, 175(6): 1551-1554.
27.	Ierullo AM, Ganapathy R, Crowley S, et al. Neonatal outcome of antenatally diagnosed congenital cystic adenomatoid malformations[J]. Ultrasound Obstet Gynecol, 2005, 26(2): 150-153.
28.	李雪, 廖松林. 成人双肺弥漫性先天性肺囊性腺瘤样畸形1例[J]. 诊断病理学杂志, 2007, 14(3): 238-239.
29.	曾骐, 冯力民, 任甄华, 等. 先天性肺囊性腺瘤样畸形的诊断与治疗[J]. 中华胸心血管外科杂志, 2003, 19(3): 148-150.

1. Ch’In KY, Tang MY. Congenital adenomatoid malformation of one lobe of a lung with general anasarca[J]. Arch Pathol(Chic), 1949, 48(3): 221-229.
2. Duncombe GJ, Dickinson JE, Kikiros CS. Prenatal diagnosis and management of congenital cystic adenomatoid malformation of the lung[J]. Am J Obstet Gynecol, 2002, 187(4): 950-954.
3. Morelli L, Piscioli I, Licci S, et al. Pulmonary congenital cystic adenomatoid malformation, typeⅠ, presenting as a single cyst of the middle lobe in an adult: case report[J]. Diagn Pathol, 2007, 2: 17.
4. Kim WS, Lee KS, Kim IO, et al. Congenital cystic adenomatoid malformation of the lung: CT-pathologic correlation[J]. AJR Am J Roentgenol, 1997, 168(1): 47-53.
5. Shanmugam G. Adult congenital lung disease[J]. Eur J Cardiothorac Surg, 2005, 28(3): 483-489.
6. Oh BJ, Lee JS, Kim JS, et al. Congenital cystic adenomatoid malformation of the lung in adults: clinical and CT evaluation of seven patients[J]. Respirology, 2006, 11(4): 496-501.
7. Dahabreh J, Zisis C, Vassiliou M, et al. Congenital cystic adenomatoid malformation in an adult presenting as lung abscess[J]. Eur J Cardiothorac Surg, 2000, 18(6): 720-723.
8. Belcher E, Lawson MH, Nicholson AG, et al. Congenital cystic adenomatoid malformation presenting as in-flight systemic air embolisation[J]. Eur Respir J, 2007, 30(4): 801-804.
9. Kim SR, Lee MH, Lee KA, et al. A 38-year-old woman with bilateral cystic lesions in both lower lung lobes[J]. Chest, 2011, 140(2): 544-548.
10. Feng A, Cai H, Sun Q, et al. Congenital cystic adenomatoid malformation of lung in adults: 2 rare cases report and review of the literature[J]. Diagn Pathol, 2012, 7: 37.
11. Khan NU, Jones MT, Greaves M. Congenital cystic adenomatoid malformation of an entire lung in a 33-year-old man: a case report and review of the literature[J]. Br J Radiol, 2008, 81(971): 276-278.
12. Cardoso WV, Itoh A, Nogawa H, et al. FGF-1 and FGF-7 induce distinct patterns of growth and differentiation in embryonic lung epithelium[J]. Dev Dyn, 1997, 208(3): 398-405.
13. Cass DL, Quinn TM, Yang EY, et al. Increased cell proliferation and decreased apoptosis characterize congenital cystic adenomatoid malformation of the lung[J]. J Pediatr Surg, 1998, 33(7): 1043-1046.
14. Cloutier MM, Schaeffer DA, Hight D. Congenital cystic adenomatoid malformation[J]. Chest, 1993, 103(3): 761-764.
15. Mas A, Mirapeix RM, Domingo C, et al. Pulmonary hypoplasia presented in adulthood as a chronic respiratory failure: report of two cases. Embryology, clinical symptoms and diagnostic procedures[J]. Respiration, 1997, 64(3): 240-243.
16. Roberts D, Sweeney E, Walkinshaw S. Congenital cystic adenomatoid malformation of the lung coexisting with recombinant chromosome 18. A case report[J]. Fetal diagn ther, 2001, 16(2): 65-67.
17. Moerman P, Fryns JP, Vandenberghe K, et al. Pathogenesis of congenital cystic adenomatoid malformation of the lung[J]. Histopathology, 1992, 21(4): 315-321.
18. Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum[J]. Hum Pathol, 1977, 8(2): 155-171.
19. Stocker J. Congenital pulmonary airway malformation-a new name for and an expanded classification of congenital cystic adenomatoid malformation of the lung[J]. Histopathology, 2002, 41(Supp1): 424-431.
20. Collins AM, Ridgway PF, Killeen RP, et al. Congenital cystic adenomatoid malformation of the lung: hazards of delayed diagnosis[J]. Respirology, 2009, 14(7): 1058-1060.
21. 常占平, 金锦善, 金妍, 等. 成人肺先天性囊性腺瘤样畸形伴发肺癌[J]. 诊断病理学杂志, 2003, 10(5): 284-286.
22. MacSweeney F, Papagiannopoulos K, Goldstraw P, et al. An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation[J]. Am J Surg Pathol, 2003, 27(8): 1139-1146.
23. Doladzas T, Arvelakis A, Karavokyros IG, et al. Primary rhabdomyosarcoma of the lung arising over cystic pulmonary adenomatoid malformation[J]. Pediatr Hematol Oncol, 2005, 22(6): 525-529.
24. 房惠琼, 李启明, 陶祥. 成人先天性肺囊性腺瘤样畸形伴类癌一例[J]. 中华病理学杂志, 2008, 37(10): 713-715.
25. Stacher E, Ullmann R, Halbwedl I, et al. Atypical goblet cell hyperplasia in congenital cystic adenomatoid malformation as a possible preneoplasia for pulmonary adenocarcinoma in childhood: a genetic analysis[J]. Hum Pathol, 2004, 35(5): 565-570.
26. Marshall KW, Blane CE, Teitelbaum DH, et al. Congenital cystic adenomatoid malformation: impact of prenatal diagnosis and changing strategies in the treatment of the asymptomatic patient[J]. AJR Am J Roentgenol, 2000, 175(6): 1551-1554.
27. Ierullo AM, Ganapathy R, Crowley S, et al. Neonatal outcome of antenatally diagnosed congenital cystic adenomatoid malformations[J]. Ultrasound Obstet Gynecol, 2005, 26(2): 150-153.
28. 李雪, 廖松林. 成人双肺弥漫性先天性肺囊性腺瘤样畸形1例[J]. 诊断病理学杂志, 2007, 14(3): 238-239.
29. 曾骐, 冯力民, 任甄华, 等. 先天性肺囊性腺瘤样畸形的诊断与治疗[J]. 中华胸心血管外科杂志, 2003, 19(3): 148-150.

West China Medical Journal

Congenital Cystic Adenomatoid Malformation of Lung in Adults

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