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Ann Thorac Surg 2007;83:687-689
© 2007 The Society of Thoracic Surgeons

Case Reports

Bronchioloalveolar Carcinoma in Congenital Cystic Adenomatoid Malformation of Lung

Douglas West, MRCSa,*, Andrew G. Nicholson, FRCPathb, Ian Colquhoun, FRCSc, James Pollock, FRCSa,c

a Department of Cardiothoracic Surgery, The Royal Hospital for Sick Children, Dalnair Street, Glasgow
b Department of Histopathology, The Royal Brompton Hospital, London
c Department of Cardiothoracic Surgery, Glasgow Royal Infirmary, Glasgow, United Kingdom

Accepted for publication June 5, 2006.

* Address correspondence to Mr West, The Royal Hospital for Sick Children, Dalnair St, Glasgow G3 8SJ, UK. (Email: dgwest{at}rcsed.ac.uk).


   Abstract
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 Abstract
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Congenital cystic adenomatoid malformation (CCAM) of lung is a rare condition with the potential for malignant transformation. We report a patient who underwent lobectomy for a cystic lung lesion, which was found to be a type 1 CCAM associated with a mucinous bronchioloalveolar carcinoma. Retrospective review of a biopsy specimen from the same lobe excised during an ipsilateral empyema drainage 11 years previously showed similar foci of bronchioloalveolar carcinoma. The patient remains well 3 years after surgery. This case demonstrates the indolent nature of malignancies seen in association with type 1 CCAMs and also that complete excision, probably by lobectomy, is the treatment of choice to avoid recurrence.


   Introduction
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 Abstract
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Congenital cystic adenomatoid malformation (CCAM) of the lung accounts for 42% of pediatric lung resections [1] and 53.9% of all lung resection for cystic disease [2] in recent series. Typically presenting antenatally or in early infancy, late presentation with lung infection is less common but well described [2]. The Stocker classification describes four types of CCAM on the basis of histopathologic appearance, but malignant transformation is principally seen in type 1 CCAMs, in which the tumor is nearly always a mucinous bronchioloalveolar carcinoma (BAC). Although well-described [3], malignant transformation is rare, probably about 1% of type 1 CCAMs.

An 18-year-old man presented with a productive cough, purulent sputum, and fever. Chest roentgenogram and computed tomography scan revealed a large cyst in the left lower lobe, for which surgical excision was advised. Nine years previously, an empyema of the inferior left hemithorax had developed. This was evacuated through a mini-thoracotomy, with full reexpansion of the lung and resolution of symptoms. At surgery, the cavity contained 200 ml of brownish gelatinous material, and a wall biopsy specimen was taken to exclude tuberculosis. During follow-up between ages 9 and 17, he remained asymptomatic and was otherwise fit and well. He was a nonsmoker.

At the time of the second procedure, thoracotomy showed virtual replacement of the left lower lobe by a cystic lesion, and a lobectomy was performed. Histology revealed a cystic mass, the lining being mainly respiratory-type epithelium characteristic of a type 1 CCAM. In addition, there was proliferation of columnar mucinous cells showing a lepidic growth pattern in adjacent alveoli, indicative of bronchioloalveolar carcinoma (Fig 1).


Figure 1
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  		Fig 1. Lobectomy specimen from 18-year-old patient. Focal bronchioalveolar carcinoma (arrow) is seen within alveolar parenchyma adjacent to the cyst wall (top). (x40 magnification, haematoxylin and eosin stain.)

 
The patient recovered well, with no evidence of locoregional or distant disease during 3 years of follow-up, but a retrospective histopathologic review of the cyst biopsy specimen 9 years previously was done in the light of these findings. This revealed a cyst abutting the visceral pleura with a similar proliferation of columnar mucinous cells both lining the cyst and extending into surrounding lung parenchyma. These findings were in keeping with BAC within a type I CCAM.


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This case is of particular interest because it shows the extremely indolent nature of BACs, as defined by the 2004 World Health Organization classification [4], in association with CCAMs. Most cases have been resected in their entirety at the time of diagnosis, with only one reported case treated conservatively. This patient had inoperable bilateral disease at presentation and progressed slowly over many years [5]. There is also a report of a mucinous BAC arising in the left lower lobe of a 19-year-old man, the patient having had an adenomatoid malformation resected from this lobe in infancy, a history not dissimilar to this case [6].

This very slow progression is reflected in data showing decreased cellular atypia, p53 expression, and proliferative activity in BACs associated with CCAM compared with de novo BAC, although this study was not statistically significant [3]. These data, together with evidence from this case and the two described previously, suggest that BAC in CCAMs are a less aggressive disease entity than BAC originating in structurally normal lung, although no comparative studies are ever likely to be available.

This case also highlights the importance of anatomic lung resection, which has been advocated for all suspected CCAMs in view of the risk of malignant transformation. This case supports this approach, as do data from the CCAM resected in infancy with BAC 19 years later where segmentectomy was the initial operation. Finally, the possibility of undiagnosed CCAM, although rare, should be considered in both children and adults presenting with repeated chest sepsis.


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  1. Shanmugan G, MacArthur K, Pollock JCS. Congenital lung malformations-antenatal and postnatal evaluation and management Eur J Cardiothorac Surg 2005;27:45-52.[Abstract/Free Full Text]
  2. Papagiannopoulos K, Hughes S, Nicholson AG, Goldstraw P. Cystic lung lesions in the pediatric and adult population: surgical experience at the Brompton Hospital Ann Thorac Surg 2002;73:1594-1598.[Abstract/Free Full Text]
  3. MacSweeney F, Papagiannopoulos K, Goldstraw P, Sheppard MN, Corrin B, Nicholson AG. An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation Am J Surg Path 2003;27:1139-1146.[Medline]
  4. Travis WD, Colby TV, Corrin B, Shimosato Y, Brambilla E. Histologic and graphical text slides for the histologic typing of lung and pleural tumorsIn: Travis WD, editor. World Health Organization Pathology Panel. WHO international histological classification of tumors. 3rd ed.. Berlin: Springer Verlag, Berlin; 1999. pp. 5.
  5. Benjamin DR, Cahill JL. Bronchioloalveolar carcinoma of the lung and congenital cystic adenomatoid malformation Am J Clin Pathol 1991;95:889-892.[Medline]
  6. Lantuejoul S, Ferretti GR, Goldstraw P, Hansell DM, Brambilla E, Nicholson AG. Metastases from bronchioloalveolar carcinomas associated with long-standing type 1 congenital cystic adenomatoid malformationsA report of two cases. Histopathology 2006;48:204-206.[Medline]



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