Correspondence

Surgery for Congenital Cystic Adenomatoid Malformation of the Lung

N Engl J Med 1996; 335:1689-1690November 28, 1996

Article

To the Editor:

In discussing Case 20-1996, which involved a congenital cystic adenomatoid malformation of the lung (June 27 issue),1 Dr. Kinane states that the diagnostic and therapeutic procedure is a resection of the abnormal tissue. This may not always be the case.

In a study of 61 live-born infants with this malformation, 16 (26 percent) did not need surgery.2 If complications or symptoms are present, then surgery is required, but if there are no symptoms, some centers recommend a policy of watchful waiting and do not subject the patient to a thoracotomy, with the removal of a segment or a lobectomy and all that this entails. Such surgery is akin to the treatment of multicystic kidney disease with prophylactic nephrectomy because of the risk of later neoplasia, an approach that has been abandoned. The evidence of a risk of cancer in congenital cystic malformation is based on a few reports3,4 and not on systematic or comprehensive data.

Simon J. Ward, M.R.C.P.
Institute for Child Health, London WC1N 1EH, United Kingdom

4 References

1. 1

   Case Records of the Massachusetts General Hospital (Case 20-1996). N Engl J Med 1996;334:1726-1732
   Full Text | Web of Science | Medline

2. 2

   Thorpe-Beeston JG, Nicolaides KH. Cystic adenomatoid malformation of the lung: prenatal diagnosis and outcome. Prenat Diagn 1994;14:677-688
   CrossRef | Web of Science | Medline

3. 3

   Heij HA, Ekkelkamp S, Vos A. Diagnosis of congenital cystic adenomatoid malformation of the lung in newborn infants and children. Thorax 1990;45:122-125
   CrossRef | Web of Science | Medline

4. 4

   Ueda K, Gruppo R, Unger F, Martin L, Bove K. Rhabdomyosarcoma of lung arising in congenital cystic adenomatoid malformation. Cancer 1977;40:383-388
   CrossRef | Web of Science | Medline

Author/Editor Response

Dr. Kinane replies:

To the Editor: Dr. Ward raises a controversial point in the management of congenital cystic adenomatoid malformation. If the findings are consistent with this disorder but there are no symptoms, should the lesion be resected? Is its neoplastic potential sufficient to warrant surgery? A recent study suggests that the lesion is due to an arrest in lung development at a premature stage, and the lesion can be reproduced in mice by overexpression of the gene for keratinocyte growth factor.1 Failure of the lung to develop from this immature state may predispose it to neoplastic changes.

Dr. Ward cites two reports of malignant change, but many reports of neoplasia developing in lung cysts appeared before 1965, when cystic lesions were less clearly classified.2 There have been reports of three cases of rhabdomyosarcoma and one of pulmonary blastoma occurring early in life in patients with congenital cystic adenomatoid malformation. These tumors are unusual complications of congenital cystic adenomatoid malformation, but bronchioloalveolar carcinoma is of particular concern. There are at least seven case reports describing the occurrence of this cancer later in life in patients with congenital cystic adenomatoid malformation.3,4 Sheffield et al. report a progression from preneoplastic to neoplastic disease when resection was delayed.5

I think there is a substantial risk of neoplasia in patients with congenital cystic adenomatoid malformation. Short-term follow-up of such patients, as described by Dr. Ward, may give the physician, and ultimately the patient, a false sense of security. I recommend surgery for congenital cystic adenomatoid malformation.

T. Bernard Kinane, M.D.
Massachusetts General Hospital, Boston, MA 02114

5 References

1. 1

   Simonet WS, DeRose ML, Bucay N, et al. Pulmonary malformation in transgenic mice expressing human keratinocyte growth factor in the lung. Proc Natl Acad Sci U S A 1995;92:12461-12465
   CrossRef | Web of Science | Medline

2. 2

   Korol E. The correlation of carcinoma and congenital cystic emphysema of the lung: report of ten cases. Dis Chest 1953;23:403-411
   Medline

3. 3

   Benjamin DR, Cahill JL. Bronchioloalveolar carcinoma of the lung and congenital cystic adenomatoid malformation. Am J Clin Pathol 1991;95:889-892
   Web of Science | Medline

4. 4

   Ribet ME, Copin MC, Soots JG, Gosselin BH. Bronchioloalveolar carcinoma and congenital cystic adenomatoid malformation. Ann Thorac Surg 1995;60:1126-1128
   CrossRef | Web of Science | Medline

5. 5

   Sheffield EA, Addis BJ, Corrin B, McCabe MM. Epithelial hyperplasia and malignant change in congenital lung cysts. J Clin Pathol 1987;40:612-614
   CrossRef | Web of Science | Medline

Citing Articles (2)

Citing Articles

1. 1

   Jean-Martin Laberge, Ioana Bratu, Hélène Flageole. (2004) The management of asymptomatic congenital lung malformations. Paediatric Respiratory Reviews 5, S305-S312
   CrossRef

2. 2

   D. Hellmuth, J.C. Glerant, H. Sevestre, A. Remond, V. Jounieaux. (1998) Pulmonary adenomatoid malformation presenting as unilobar cysts in an adult. Respiratory Medicine 92:12, 1364-1367
   CrossRef