Congenital cystic adenomatoid malformation of lung in adults: 2 rare cases report and review of the literature

Abstract

Congenital cystic adenomatoid malformation (CCAM), also named congenital pulmonary airway malformation (CPAM), is a congenital abnormality of lung which is uncommon in adults. Here we present 2 adult cases of CCAM with unusual clinical and pathologic findings. One case was complicated with aspergillosis which was seldom reported. The other case was suffered bilateral lesions and the patient's mother had been previously radiographically discovered bilateral cystic lesions that CCAM could not be ruled out. A review of currently published related literatures has also been provided.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/6406766736634578.

Congenital Cystic Adenomatoid Malformation: Case Report

“…Postnatal and in adults patients, lobectomy is the treatment of choice for symptomatic cases. [14,[18][19][20] However, there is a controversy whether or not all cases be subjected to surgery. Surgery is indicated for the following reasons: (i) definite histological diagnosis, (ii) history of recurrent infections, and (iii) risk of malignancies which have been rarely reported.…”

The telltale Cinderella story of Congenital cystic adenomatoid malformation of the lungs in a child: a rare case report from DAKSHIN KANNADA

“…1 They constitue the category of bronchopulmonary foregut malformations .The disorder is often referred to by another name, congenital pulmonary airway malformation (CPAM). In this anomaly an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue.…”

“…All types are intrinsically the same lesion, which differ on the radiological presentation. Type I CCAM is characterized by single or multiple cysts more than 3 cm in diameter lined by pseudo stratified ciliated columnar epithelium, along with mucous cells which are considered to be potentially mutant to adenocarcinoma 1,19 . Type II lesion is consisted of multiple terminal bronchiolar-like uniform cysts less than 2 cm in IJBR (2013) 04 (08) www.ssjournals.com diameter, lined by cuboidal to columnar epithelium.…”

“…1,2 It was classified into 3 subtypes in 1977 3 , and expanded into 5 types with a new name as congenital pulmonary airway malformation (CPAM) by Stocker in 2002. 4 80% to 85% of cases are recognized in the first 2 years of life, adult presentation is uncommon 5 .…”

Congenital cystic adenomatoid malformation of lung in adult complicated by aspergilloma presenting as recurrent hemoptysis- 3 rd case report in world literature

“…By far bacterial infection is the most frequent reported CCAM complication which causes acute fever and lung abscess [5]. To our knowledge only 2 cases are reported to have aspergilloma as a complication of CCAM [6,7] . Aspergillus primarily affects the lungs, causing 4 main syndromes: 1) allergic bronchopulmonary aspergillosis [ABPA], 2) chronic necrotizing Aspergillus pneumonia also termed chronic necrotizing pulmonary aspergillosis [CNPA], 3) aspergilloma, and 4) invasive aspergillosis.…”