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Eur J Cardiothorac Surg 2000;18:720-723
© 2000 Elsevier Science NL

Case report

Congenital cystic adenomatoid malformation in an adult presenting as lung abscess

^a Thoracic Surgery Department, Saint Savas Anticancer Hospital, Athens, Greece
^b Chest Medicine Department, Saint Savas Anticancer Hospital, Athens, Greece
^c Pathology Department, Saint Savas Anticancer Hospital, Athens, Greece

Received 23 May 2000; received in revised form 15 August 2000; accepted 19 September 2000.

Corresponding author. Parodos Helmou, 145 65 Agios Stefanos, Attiki, Greece Tel.: +30-1-814-1178; fax: +30-1-642-1022/0146
e-mail: dkakats{at}cc.uoa.gr

	Abstract

Top Abstract 1. Case report 2. Comment References

 
The case of a 21-year-old male with congenital cystic adenomatoid malformation is presented. His medical history started after his birth with recurrent pulmonary infections during his infancy. Lung abscess of the right lower lobe was suspected and right lower lobectomy was performed to remove a sizeable mass infiltrating the largest part of the lobe. The clinical features, diagnostic procedures, differential diagnosis, pathologic characteristics, therapeutic assessment, etiopathology and prognosis of the tumor are discussed.

Key Words: Bronchopulmonary foregut malformations • Fetal masses • Congenital malformation • Surgical resection • Lung abscess • Recurrent pulmonary infections

	1. Case report

Top Abstract 1. Case report 2. Comment References

 
Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare benign tumor first described by Chin and Tang in 1949 [1]. According to Hellmuth et al. [2] until 1998 only 23 cases of CCAM in adulthood have been reported in the literature.

A 21-year-old male was admitted to our department because of a sizeable infiltration on chest X-ray located in the posterior basic segment of the right lower lobe. The patient at the admission was asymptomatic, while 1 year before his admission, persistent non-productive cough was a prominent symptom. Other clinical and laboratory signs of infection were absent. The finding was attributed to a chronic abscess of the right lung from his childhood because of his past medical history which started 1 month after his birth with respiratory distress and recurrent pulmonary infections with fever 38–38.5°C during his infancy. At that time, all X-rays of the chest showed a non-homogenous opacity of the right lung. These radiological findings were confirmed in every subsequent investigation interpreted as fibrous elements of the right pulmonary base indicative of an inflammatory lesion.

At his last admission the lesion was detected on X-ray examination and computed tomography (CT)-scan (Fig. 1) as excavated tumor whereas bronchoscopy was negative and lung-function study was extremely satisfactory with values superior to the predictable (forced vital capacity (FVC): 6.34–118%, forced expiratory volume in 1 s (FEV1): 5.46–120%). Cardiac function was proved to be normal, while no other pathology was mentioned.

View larger version (94K): [in this window] [in a new window]   Fig. 1. Preoperative CT-scan with the lesion appearing as a lung abscess of the right lower lobe.

Macroscopic examination revealed a tumor sized 5.5x6x4 cm located in a distance of 1.5 cm from the limit of the excision, not encapsulated but well confined from the pulmonary parenchyma around it, solid with sites of cavity formation full of gluish colorless material. The tumor was extended up to the visceral pleura while anatomic relation with bronchial lumen did not exist. Histologically the tumor was consisted of cysts lined by tall columnar mucous epithelium (Fig. 2a) . Cuboidal or flattened mucus-secreting cells lined some glandular structures. In areas, the structures were dilated and filled by mucus (Fig. 2b). In other areas, cells were heaped into tufts or papillae showing mild cytological atypia and stratification. There were not identified areas with nuclear pleomorphism, many mitoses or necrosis. The surrounding stroma showed mild inflammation. All mucin stains were strongly positive.

View larger version (114K): [in this window] [in a new window]   Fig. 2. (a) Tumor consisted of structures lined with tall columnar mucinous epithelium (HSE x10). (b) Cystic dilatation of the structures filled with mucus (HSE x10).

The post-operative course was uneventful and the patient is doing well 3 years after the operation.

	2. Comment

Top Abstract 1. Case report 2. Comment References

 
CCAM of the lung is a rare developmental abnormality of the lung and it has no sex predilection while female predominance has been also reported. It is usually unilateral and sublobar or lobar in size, but occasionally it can be multilobar.

Adenomatoid cystic malformations of the lung are considered bronchopulmonary foregut malformations caused probably by an arrest in lung development between 4th and 7th week of fetal life [4]. CCAM type 1, showing a bronchiolar type of epithelium, as also types 2 and 3, arise from an insult at the branching of the bronchopulmonary tree in the pseudoglandular stage; CCAM type 4, with an acinar-alveolar type of epithelium, originates in saccular stage [4].

A number of congenital masses diagnosed on prenatal sonograms, resolve partially or completely on sonograms performed late in gestation. It has been demonstrated that children with ‘disappearing’ fetal lung masses have persistent abnormalities after birth that are often subtle on radiographs but are well shown with CT [5]. In our case, a recession of symptoms has been observed after the first year of life and our patient remained nearly asymptomatic during his childhood and adolescence, while the pathology existed as a slight opacity in the right lower pulmonary field.

The clinical features concerning CCAM may be variant, but repeated pulmonary infections [6] is the main clinical finding. Bronchoscopic examination is usually normal. Chest X-rays and CT-scan may reveal an opacity in the pulmonary zone affected, but generally the different studies performed do not step forward the diagnostic procedure. High-resolution CT (HRCT) is proposed as a reliable paraclinical investigative procedure which allows accurate assessment of lesion site and extent. Differential diagnosis has to be done from other rare cystic tumors.

Malignant potentials of these tumors have been recognized. Development of bronchoalveolar carcinoma [7] and a case of embryonal pulmonary rhabdomyosarcoma (RMS) within a CCAM [8] have been described. Although the etiologic factors predisposing infants and children to pulmonary neoplasms are unknown, pulmonary developmental abnormalities are likely to play a pathogenetic role. It remains to be determined, however, if these associations may be considered as special pathologic entities in the context of clinical syndromes.

CCAM may be confused on X-ray examination with lung infection while the possibility of an infected tumor cannot be ruled out. After treatment of the diagnosed pulmonary infection, the radiological appearance of the CCAM may be changed in a lacent, cystic, fluid-filled mass resembling an abscess [9]. It is characteristic that the preoperative diagnosis of the case, according to X-ray and CT-findings, was chronic lung abscess or neoplasm centrally excavated. Consequently, the risk of pulmonary compression, infection or malignant degeneration make surgical exploration imperative, even in asymptomatic patients [10]. The prognosis after radical excision of the tumor seems to be excellent even when a transition into a bronchioloalveolar carcinoma is revealed by chance.

	References

Top Abstract 1. Case report 2. Comment References

 

1. Ch'in K.Y., Tang M.Y. Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol Lab Med 1949;48:221-229.
2. Hellmuth D., Glerant J.C., Sevestre H., Remond A., Jounieaux V. Pulmonary adenomatoid malformation presenting as unilobar cysts in an adult. Respir Med 1998;92:1364-1372.[Medline]
3. Stocker J.T. Congenital and developmental diseases. In: Dali D.H., Hammar S.P., eds. Pulmonary pathology, 2nd edition New York: Springer–Verlag, 1994:155-190.
4. Morotti R.A., Cangiarella J., Gutierrez M.C., Jagirdar J., Askin F., Singh G., Profitt S.A., Wert S.E., Whitsett J.A., Greco M.A. Congenital cystic adenomatoid malformation of the lung (CCAM): evaluation of the cellular components. Hum Pathol 1999;30:618-625.[Medline]
5. Winters W.D., Effmann E.L., Nghiem H.V., Nyberg D.A. Disappearing fetal lung masses: importance of postnatal imaging studies. Pediatr Radiol 1997;27:535-539.[Medline]
6. Lackner R.P., Thompson A.B., 3rd, Rikkers L.F., Galbraith T.A. Cystic adenomatoid malformation involving an entire lung in a 22-year old woman. Ann Thorac Surg 1996;61:1827-1829.[Abstract/Free Full Text]
7. Kaslovsky R.A., Purdy S., Dangman B.C., McKenna B.J., Brien T., Ilves R. Bronchioloalveolar carcinoma in a child with congenital cystic adenomatoid malformation. Chest 1997;112:548-551.[Abstract/Free Full Text]
8. d'Agostino S., Bonoldi E., Dante S., Meli S., Cappellari S., Musi L. Embryonal rhabdomyosarcoma of the lung arising in cystic adenomatoid malformation: case report and review of the literature. J Pediatr Surg 1997;32:1381-1383.[Medline]
9. Kieffer F., Ferriere A., Magny J.F., Coatantiec Y., Revillon Y., Voyer M. Cystic adenomatoid malformation of the lung revealed in a newborn infant by an image of a lung abscess. Arch Pediatr 1996;3:470-472.[Medline]
10. Evrard V., Ceulemans J., Coosemans W., De Baere T., De Leyn P., Deneffe G., Devlieger H., De Boeck C., Van Raemdonck D., Lerut T. Congenital parenchymatous malformations of the lung. World J Surg 1999;23:1123-1132.[Medline]

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