Background: Congenital cystic adenomatoid malformation of the lung (CCAM) is an embryonic developmental anomaly of an unknown etiology usually diagnosed antenatally by imaging techniques. A minority of cases may not be identified by prenatal imaging techniques and may go unnoticed for the first 6 months of their extrauterine life. Due to its rarity, physicians are unlikely to suspect the condition. Objectives: To highlight the embryology, clinical symptomatology, diagnostic procedures, therapeutic approach and clinical follow-up of a series of 12 patients with late-onset CCAM. Methods: An observational study which offers the description of the clinical presentation, diagnostic methods, treatment and follow-up of 12 patients with late-onset CCAM. Setting: A 600-bed teaching hospital in a reference area of 350,000 inhabitants. Patients: 12 patients from 1983 to 1999. Results: Twelve diagnosed cases of late-onset CCAM. Mean age at diagnosis: 6.7 years (range: 6 months to 23 years). Clinical presentation: 9 out of 12 (75%) with repeated lung infections, 2 out of 12 (16%) chance finding, and 1 case (8%) with pneumothorax. On pathological examination, 7 were found to be CCAM type I and 4 CCAM type II according to Stocker’s classification; 1 patient is currently awaiting surgery. The diagnostic method of choice nowadays is a computed tomography (CT) scan performed in the 7 more recent cases; in the former 5 cases an isotopic lung scan was done (and in 2 of them a bronchography was also performed). Treatment: 11 patients were operated: 8 lobectomies, 2 segmentectomies and 1 localized resection. Mean follow-up: 8 years (range: 6 months to 16 years). Complications: One reintervention due to a reappearance of the lesion in the patient who underwent localized resection of the CCAM. No cases of malignancy were found. Conclusions: Late-onset CCAM is an infrequent illness which requires a high level of clinical suspicion. It usually presents in the form of repeated infections. The most frequent pathological forms are type I and II (Stocker). The diagnostic method of choice is the CT scan. The recommended treatment is radical surgery of the lesion once diagnosis has been established. Malignancy and relapses are very infrequent when radical surgery is not postponed.

1.
Ch’in KY, Tang MY: Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol Lab Med 1949;48:221–229.
2.
Stocker JT, Madwell JE, Drake RM: Congenital cystic adenomatoid malformation of the lung. Hum Pathol 1977;8:156–171.
3.
Conran RM, Stocker JT: Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation type 2: Report of 50 cases. Pediatr Dev Pathol 1999;2:454–463.
4.
Moerman P, Fryns JP, Vandenberghe K, et al: Pathogenesis of congenital cystic adenomatoid malformation of the lung. Histopathology 1992;21:315–321.
5.
Dommergues M, Louis-Sylvestre C, Mandelbrot L, et al: Congenital adenomatoid malformation of the lung: When is active fetal therapy indicated? Am J Obstet Gynecol 1997;197:953–958.
6.
Adzick NS, Harrison MR, Crombleholme TM, et al: Fetal lung lesions: Management and outcome. Am J Obstet Gynecol 1998;179:884–889.
7.
Bailey PV, Tracy T, Connors RH, et al: Congenital bronchopulmonary malformations: Diagnostic and therapeutic considerations. Thorac Cardiovasc Surg 1990;99:597–603.
8.
Haddon MJ, Bowen A: Bronchopulmonary and neurenteric forms of foregut anomalies: Imaging for diagnosis and management. Radiol Clin North Am 1991;29:241–254.
9.
Cloutier MM, Schaeffer DA, Hight D: Congenital cystic adenomatoid malformation. Chest 1993;103:761–764.
10.
Cangiarella J, Greco MA, Askin F, et al: Congenital cystic adenomatoid malformation of the lung: Insights into the pathogenesis utilizing quantitative analysis of vascular marker CD34 (QBEND-10) and cell proliferation marker MIB-1. Mod Pathol 1995;8:913–918.
11.
Cha I, Adzick NS, Harrison MR, et al: Fetal congenital cystic adenomatoid malformations of the lung: A clinicopathologic study of eleven cases. Am J Surg Pathol 1997;21:537–544.
12.
Alescio T, Cassini A: Induction in vitro of tracela buds by pulmonary mesenchyme grafted on tracela epithelium. J Exp Zool 1962;150:83–94.
13.
Zou H, Niswander L: Requirement of BMP signaling in interdigital apoptosis and scale formation. Science 1996;272:738–741.
14.
Cardoso WV, Itoh A, Nogawa H, et al: FGF-1 and FGF-7 induce distinct patterns of growth and differentiation in embryonic lung epithelium. Dev Dyn 1997;208:398–405.
15.
Cass DL, Quinn TM, Yang EY, et al: Increased cell proliferation and decreased apoptosis characterize congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 1998;33:1043–1047.
16.
Mas A, Mirapeix RM, Domingo C, et al: Pulmonary hypoplasia presented in adulthood as a chronic respiratory failure: Report of two cases. Respiration 1997;64:240–243.
17.
Van Leeuwen K, Teitelbaum DH, Hirschl RB, et al: Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications and natural history. J Pediatr Surg 1999;34:794–799.
18.
Waszak P, Claris O, Lapillone A, et al: Cystic adenomatoid malformation of the lung: Neonatal management of 21 cases. Pediatr Surg Int 1999;15:326–331.
19.
Cacciari A, Ceccarelli PL, Pilu GL, et al: A series of 17 cases of congenital cystic adenomatoid malformation of the lung: Management and outcome. Eur J Pediatr Surg 1997;7:84–89.
20.
Sakala EP, Perrott WS, Grube GL: Sonographic characteristics of antenatally diagnosed extralobar pulmonary sequestration and congenital cystic adenomatoid malformation. Obstet Gynecol Surv 1994;49:647–655.
21.
Winter WD, Effmann EL, Ngiem HV, et al: Disappearing fetal lung masses: Importance of postnatal imaging studies. Pediatr Radiol 1997;27:535–539.
22.
Sapin E, Lejeune V, Barbet JP, et al: Congenital adenomatoid disease of the lung: Prenatal diagnosis and perinatal management. Pediatr Surg Int 1997;12:126–129.
23.
Lackner RP, Thompson AB, Rikkers LF, et al: Cystic adenomatoid malformation involving an entire lung in a 22-year-old woman. Ann Thorac Surg 1996;61:1827–1829.
24.
Jaby O, Larroquet M, Balquet P, et al: Cystic adenomatoid malformation of the lung. Pediatr Pulmonol 1997(suppl 16):131.
25.
Canals J, Boix J, Peiro JL, et al: Congenital cystic adenomatoid malformation of the lung. Presentation of 26 cases. Cir Pediatr 1994;7:92–96.
26.
Lejeune C, Deschildre A, Thumerelle C, et al: Spontaneous pneumothorax revealing cystic adenomatoid malformation of the lung in a 13-year-old child. Arch Pediatr 1999;6:863–866.
27.
Bentur L, Canny G, Thorner P, et al: Spontaneous pneumothorax in cystic adenomatoid malformation. Unusual clinical and histologic features. Chest 1991;99:1292–1293.
28.
Alter SJ: Spontaneous pneumothorax in infants: A 10-year review. Pediatr Emerg Care 1997;13:401–403.
29.
Spence LD, Ahmed S, Keohane C, et al: Acute presentation of cystic adenomatoid malformation of the lung in a 9-year-old child. Pediatr Radiol 1995;25:572–573.
30.
Patz EF, Muller NL, Swensen SJ, et al: Congenital cystic adenomatoid malformation in adults. CT findings. J Comput Assist Tomogr 1995;3:361–364.
31.
Heij HA, Ellelkamp S: Diagnosis of congenital cystic adenomatoid malformation of the lung in newborn infants and children. Thorax 1990;45:122–125.
32.
Granata C, Gambini C, Balducci T, et al: Bronchioloalveolar carcinoma arising in a congenital cystic adenomatoid malformation in a child: A case report and review of the malignancies originating in CCAM. Pediatr Pulmonol 1998;25:62–66.
33.
Frenckner B, Freyschuss U: Pulmonary function after lobectomy for congenital lobar emphysema and congenital cystic adenomatoid malformation. A follow-up study. Scand J Thorac Cardiol Surg 1982;16:293–298.
34.
Browdie D, Todd D, Agnew R, et al: The use of nonanatomic resection in infants with extensive congenital adenomatoid malformation of the lung. J Thorac Cardiovasc Surg 1993;105:732–736.
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