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Congenital cystic adenomatoid malformation of lung: Report of two cases with review of literature.

Chikkannaiah P, Kangle R, Hawal M - Lung India (2013)

Bottom Line: Congenital cystic adenomatoid malformations (CCAM) also known as congenital pulmonary airway malformation is a developmental, non-hereditary, hamartomatous abnormality of lung with unknown etiology.It is a rare disease with an incidence of 1 in 25,000 to 1 in 35,000.It is a disease of infancy with most of the cases diagnosed within first 2 years of life.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Karnataka Lingayat Education University's Jawaharlal Nehru Medical College, Belgaum, Karnataka, India.

ABSTRACT
Congenital cystic adenomatoid malformations (CCAM) also known as congenital pulmonary airway malformation is a developmental, non-hereditary, hamartomatous abnormality of lung with unknown etiology. It is a rare disease with an incidence of 1 in 25,000 to 1 in 35,000. It is a disease of infancy with most of the cases diagnosed within first 2 years of life. We report autopsy findings of two fetuses with congenital cystic adenomatoid malformation (Stocker Type II and I) with brief review of literature.

No MeSH data available.


Related in: MedlinePlus

(a) Gross photo of resected lungs showing enlargement of right lung. (b and c) Microscopy showing large cyst (bronchiole like) surrounded by small cysts, lined by stratified columnar epithelium and having thin fibromuscular septa (hematoxylin and eosin (H and E), ×100). (d) High power of the large cyst showing lining epithelium (H and E, ×400)
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Figure 1: (a) Gross photo of resected lungs showing enlargement of right lung. (b and c) Microscopy showing large cyst (bronchiole like) surrounded by small cysts, lined by stratified columnar epithelium and having thin fibromuscular septa (hematoxylin and eosin (H and E), ×100). (d) High power of the large cyst showing lining epithelium (H and E, ×400)

Mentions: On autopsy, it was a male fetus weighing 1,450 g. External examination did not reveal any anomaly. On internal examination all organs were in situ. The right lung was enlarged (7 × 5 × 3 cm) cut-section showed cysts of varying sizes majority of them were < 2 cm. The left lung was normal (3.5 × 2 × 1 cm). On histology, right lungs showed multiple cysts relatively uniform in size resembling bronchioles, lined by stratified columnar epithelium, and having thin fibromuscular septa. Mucus production was not evident on periodic acid Schiff (PAS) staining [Figure 1a-d]. All other visceral organs were congested, but well-developed and revealed no anomalies. With the above features, a pathological diagnosis of CCAM Stocker type II was offered.


Congenital cystic adenomatoid malformation of lung: Report of two cases with review of literature.

Chikkannaiah P, Kangle R, Hawal M - Lung India (2013)

(a) Gross photo of resected lungs showing enlargement of right lung. (b and c) Microscopy showing large cyst (bronchiole like) surrounded by small cysts, lined by stratified columnar epithelium and having thin fibromuscular septa (hematoxylin and eosin (H and E), ×100). (d) High power of the large cyst showing lining epithelium (H and E, ×400)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3775204&req=5

Figure 1: (a) Gross photo of resected lungs showing enlargement of right lung. (b and c) Microscopy showing large cyst (bronchiole like) surrounded by small cysts, lined by stratified columnar epithelium and having thin fibromuscular septa (hematoxylin and eosin (H and E), ×100). (d) High power of the large cyst showing lining epithelium (H and E, ×400)
Mentions: On autopsy, it was a male fetus weighing 1,450 g. External examination did not reveal any anomaly. On internal examination all organs were in situ. The right lung was enlarged (7 × 5 × 3 cm) cut-section showed cysts of varying sizes majority of them were < 2 cm. The left lung was normal (3.5 × 2 × 1 cm). On histology, right lungs showed multiple cysts relatively uniform in size resembling bronchioles, lined by stratified columnar epithelium, and having thin fibromuscular septa. Mucus production was not evident on periodic acid Schiff (PAS) staining [Figure 1a-d]. All other visceral organs were congested, but well-developed and revealed no anomalies. With the above features, a pathological diagnosis of CCAM Stocker type II was offered.

Bottom Line: Congenital cystic adenomatoid malformations (CCAM) also known as congenital pulmonary airway malformation is a developmental, non-hereditary, hamartomatous abnormality of lung with unknown etiology.It is a rare disease with an incidence of 1 in 25,000 to 1 in 35,000.It is a disease of infancy with most of the cases diagnosed within first 2 years of life.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Karnataka Lingayat Education University's Jawaharlal Nehru Medical College, Belgaum, Karnataka, India.

ABSTRACT
Congenital cystic adenomatoid malformations (CCAM) also known as congenital pulmonary airway malformation is a developmental, non-hereditary, hamartomatous abnormality of lung with unknown etiology. It is a rare disease with an incidence of 1 in 25,000 to 1 in 35,000. It is a disease of infancy with most of the cases diagnosed within first 2 years of life. We report autopsy findings of two fetuses with congenital cystic adenomatoid malformation (Stocker Type II and I) with brief review of literature.

No MeSH data available.


Related in: MedlinePlus