Pulmonary congenital cystic adenomatoid malformation, type I, presenting as a single cyst of the middle lobe in an adult: case report
1 Department of Pathology, "S. Maria del Carmine" Hospital, Rovereto (TN), Italy
2 Department of Radiology, Civil Hospital of Budrio (BO), Italy
3 Department of Pathology, "National Institute for Infectious Diseases – L. Spallanzani" IRCCS, Rome, Italy
4 Department of Radiology, Civil Hospital of Bentivoglio (BO), Italy
5 Department of Radiology, "S. Salvatore" Hospital, L'aquila, Italy
6 Department of Pathology, Istituto Nazionale per le Malattie Infettive (INMI) "Lazzaro Spallanzani", IRCCS, Via Portuense, 292 00149 Roma, Italy
Diagnostic Pathology 2007, 2:17 doi:10.1186/1746-1596-2-17Published: 7 June 2007
Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon fetal development anomaly of the terminal respiratory structures. The large cyst type usually occurs in stillborn infants or newborn infants with respiratory distress. Cases of CCAM have been previously described in adulthood, more often type I with multiloculated cystic lesions.
We report a case of type I CCAM presenting as a single, expansive cystic mass in the middle pulmonary lobe in a 38-year-old man, revealed by persistent cough and haemoptysis. Computed tomographic scan showed a single cyst with air fluid level, occupying the lateral segment of the lobe. When the type I CCAM is a single cyst, other cystic pulmonary lesions must be excluded. The intrapulmonary localization and the absence of cartilage in the cyst wall are conclusive findings of CCAM. The pathogenesis, management and differential diagnosis with other lung malformations are discussed along with a review of the literature.
The literature data confirm that surgical resection is the treatment of choice in all cases of CCAM and in the cases of cystic pulmonary lesions with uncertain radiological findings, in order to perform a histological examination of the lesion and to prevent infection and the potential neoplastic transformation.