BRONCHIOLOALVEOLAR CARCINOMA ARISING IN CONGENITAL CYSTIC ADENOMATOIDMALFORMATION IN A CHILD - A CASE-REPORT AND REVIEW ON MALIGNANCIES ORIGINATING IN CONGENITAL CYSTIC ADENOMATOID MALFORMATION

A type I congenital cystic adenomatoid malformation (CCAM) in the left lower lobe was removed from a 11-year-old boy with a 3-month history of recurrent pneumonia. As incidental finding, a bronchioloalveolar ca rcinoma (BAC) was found in the lung parenchyma adjacent to the cyst. A left lower lobectomy was performed. At 18 months after surgery the pa tient is well and free of neoplastic disease. To the best of our knowl edge, this association has not been reported previously in a pediatric patient. Malignancies complicating CCAM are rarely seen, but have bee n reported in adults. Including our case, eight cases of BAC and five cases of rhabdomyosarcoma (RMS) in association with CCAM have been rep orted so far. As CCAM can host metaplastic mucous cells, primitive mes enchymal cells and differentiated but poorly organized striated muscle fibers, it has been proposed that CCAM may act as a predisposing cond ition for oncogenesis. Our experience adds further support that CCAM c an act as a premalignant lesion. Previous reports of both BAC and RMS in asymptomatic CCAM suggest prompt resection shortly after diagnosis. (C) 1998 Wiley-Liss, Inc.