congenital cystic adenomatoid malformation


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congenital cystic adenomatoid malformation

A rare developmental defect of the lungs seen primarily in infants, which displays both immaturity and malformation of the small airways and parenchyma distal to it. Pulmonary sequestration can be difficult to distinguish from congenital cystic adenomatoid malformation (CCAM), but the presence of a feeding artery from the systemic circulation is helpful.

Types
Type 0: Very rare, incompatible with life; presents as a solid mass containing bronchial-like structures and abundant cartilage.
Type 1: Most common type; contains large, multilocular cysts with broad fibrous septa, mucinous cells and focal cartilage.
Type 2: Contains small, cystically dilated bronchiolar structures (usually <2.0 cm) lined by respiratory epithelium; wall may contain striated muscle.
Type 3: Presents as a solid lesion with minute cysts (usually <0.2 cm) lined by cuboidal epithelium, surrounded by immature, fetal-type parenchyma.
Type 4: Has large cysts that are peripheral and are lined by type-1 pneumocytes and low columnar epithelium and have hypercellular stroma.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.

Congenital cystic adenomatoid malformation (CCAM)

A condition in which one or more lobes of the fetal lungs develop into fluid-filled sacs called cysts.
Mentioned in: Prenatal Surgery
Gale Encyclopedia of Medicine. Copyright 2008 The Gale Group, Inc. All rights reserved.
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References in periodicals archive ?
Long-term outcome of congenital cystic adenomatoid malformation. Pediatr Surg Int 1999;15:332-5.
Formerly termed congenital cystic adenomatoid malformation, this lesion is now more aptly called congenital pulmonary airway malformation (CPAM) to encompass the gamut of histological appearances.
El Koraichi, "Congenital cystic adenomatoid malformation --Dangers of misdiagnosis: A case report," Journal of Medical Case Reports, vol.
These cases occurred in an 11-month-old male with congenital cystic adenomatoid malformation of the right lung [8] and in 4 patients with congenital renal anomalies (a child with contralateral renal agenesis, a teenage female with an extrarenal pelvis kidney, a teenage female with a horseshoe kidney, and a young male with ipsilateral renal agenesis) [9-11].
Introduction: Congenital cystic adenomatoid malformation occurs due to non-functional solid lung tissue formed by bronchioalveolar maturation stopping and mesenchymal cells over multiplying in the fourth-eighth week of pregnancy.
Based on the clinical and radiological findings, differential diagnosis of a duplication cyst or congenital cystic adenomatoid malformation was suggested.
(i) Congenital Pulmonary Airway Malformation (CPAM), Formally Congenital Cystic Adenomatoid Malformation (CCAM).
Histopathological examination showed congenital cystic adenomatoid malformation of lung.
Atypical goblet cell hyperplasia in congenital cystic adenomatoid malformation as a possible preneoplasia for pulmonary adenocarcinoma in childhood: a genetic analysis.
Congenital cystic diseases of the lung and mediastinum include congenital cystic adenomatoid malformation (CCAM), bronchopulmonary "sequestration", congenital lobar emphysema, broncogenic cyst, esophageal duplication cysts and neurogenic cysts (1).
Congenital cystic adenomatoid malformation (CCAM) of the lungs is a developmental abnormality (hamartoma) (1).

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