symptoms may be related to disseminated disease, such as bone pain or weight. As a subtype of adenocarcinoma, BAC exhibits an excellent prognosis when v2 cm in diameter at the time of diagnosis. Indeed, there is almost 100% 5-yr survival under these circumstances [14]. Does CPAM type 1 with mucinous foci predispose to BAC? In recent years, the question of the association of CPAM type 1 with BAC has been raised on the basis of case reports of BAC having occurred in older children who, as infants, had had CPAM type 1 partially or completely resected. The first such report, in 1991, was that of Benjamin and Cahill [15], followed in 1995 by that of Ribet et al. [16]. In 1998, Ota et al. [17], having previously noted gastric mucins in mucinous BAC, studied 12 out of 26 cases of CPAM type 1 containing mucous cells and found mucins similar to those of the mucinous BACs. This association has been supported by the finding of similar genetic abnormalities (including gains in chromosomes 2 and 4) in both CPAM type 1 goblet cells and the cells of BAC [18]. Most recently, Ioachimescu and Mehta [14] described the 15-yr course of the development of a BAC (diagnosis at age 6 yrs, along with CPAM type 1), which subsequently developed into an invasive adenocarcinoma. They emphasised the unique feature of a lesion that may exhibit lack of growth for years before becoming an aggressive lesion and suggest the interim phase of atypical adenomatous hyperplasia between the CPAM-1 and BAC before it becomes an invasive adenocarcinoma. Fig. 7. Congenital pulmonary airway malformation type 4. Cut section of the lung displaying multiple large cysts with thin walls traversed by areas of density representing vessels in the walls. J.T. STOCKER, A.N. HUSAIN 12
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