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Congenital Cystic Lung Lesions

Redefining the Natural Distribution of Subtypes and Assessing the Risk of Malignancy

Pogoriler, Jennifer, MD, PhD*; Swarr, Daniel, MD; Kreiger, Portia, MD*; Adzick, N. Scott, MD; Peranteau, William, MD

The American Journal of Surgical Pathology: January 2019 - Volume 43 - Issue 1 - p 47–55
doi: 10.1097/PAS.0000000000000992
Original Articles

Asymptomatic cystic lung lesions—congenital pulmonary airway malformations (CPAMs), sequestrations, and bronchogenic cysts—are commonly diagnosed prenatally. Indications to resect are to eliminate risk of malignancy or infection. CPAMs consist of a spectrum of malformations, with type 1 historically considered the most common. Mucinous cell clusters, seen almost exclusively in type 1, are premalignant lesions at risk for progression to mucinous adenocarcinoma. We reviewed and classified 2.5 years of consecutive, prenatally diagnosed lesions as extralobar sequestration, intralobar sequestration, type 1 CPAM, type 2 CPAM/bronchial atresia, or “other” to determine the distribution of lesion types and risk of malignancy. One hundred eighty-four lesions in 174 patients showed type 1 CPAM to be least common subtype. Type 1 CPAMs had more severe presentation, infrequently had features of obstruction, and usually had cysts ≥2 cm. Fifteen of eighteen type 1 CPAMs had mucinous cell clusters (total risk, 8%), with mucous cells outside main cyst in 12/15. No pleuropulmonary blastomas were identified. Additional historic cases were reviewed to further evaluate risk of malignancy. Over 14 years, 28 infants with fetal/type 1 lesions were identified, with clusters of mucinous cells in 75% of cases. A total of 9 pleuropulmonary blastomas were diagnosed in 6 patients over 16 years. Contrary to historical studies, type 1 CPAMs are much less common than type 2, likely related to detection of asymptomatic lesions prenatally. A majority of type 1 CPAMs contain mucinous cell clusters. This data is useful in management of patients in centers that do not resect asymptomatic lesions.

Departments of *Pathology and Laboratory Medicine

Surgery, Children’s Hospital of Philadelphia, Philadelphia, PA

Division of Neonatology & Pulmonary Biology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Jennifer Pogoriler, MD, PhD, Department of Pathology and Laboratory Medicine, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, 5th Floor, Main Building Suite, 5NW13, Philadelphia, PA 19104 (e-mail:

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