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Congenital Cystic Adenomatoid Malformation of the Lung. Classification and Morphologic Spectrum

J. Thomas Stocker, JE Madewell, RM Drake

202396 citationsDOI

Abstract

This chapter describes thirty-eight cases of congenital cystic adenomatoid malformation (CCAM) of the lung and proposes a classification based on clinical, gross, and microscopic criteria. It also describes histological attributes of three types. Type I lesions being single or multiple large cysts lined by ciliated pseudostratified columnar epithelium. The walls of these cysts contain prominent smooth muscle, elastic tissue and mucus-producing cells. Type II lesions are composed of multiple small cysts lined by ciliated cuboidal to columnar epithelium. Type III lesions are large, bulky and noncystic. Bronchiole-like structures are lined by ciliated cuboidal epithelium. The chapter then describes 38 cases taken from the Armed Forces Institute of Pathology in Washington from as far back as 1917 to 1975. Up until this point, only something like 70 cases of CCAM had been published in the literature since its first acknowledged description in 1949, so this represents a huge part of existing knowledge to that point and quickly became definitive.

Topics & Concepts

Congenital Cystic Adenomatoid MalformationLungMedicinePathologyBiologyInternal medicineGeneticsPregnancyFetusCongenital Diaphragmatic Hernia StudiesTracheal and airway disordersNeonatal Respiratory Health Research
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