Expression of epidermal growth factor receptor, but not K-RAS mutations, is present in congenital cystic airway malformation/congenital pulmonary airway malformation

Hua Guo, Mariana M. Cajaiba, Dariusz Borys, Maria C. Gutierrez, Herman Yee, Rosa M. Drut, Ricardo Drut, Frederic Askin, Miguel Reyes-Múgica, M. Alba Greco

Research output: Contribution to journalArticle

14 Scopus citations

Abstract

Congenital cystic airway malformation/congenital pulmonary airway malformation (CCAM/CPAM) of the lung is a rare but well-described malformative lesion of pulmonary parenchyma characterized by the abnormal maturation of airways along with an increase in terminal respiratory structures, resulting in cysts of variable sizes. Five types have been classified based on morphological analysis. Although the etiology of the lesion is still unclear, recent data suggest that bronchial atresia is a predisposing/associated anomaly. A described association between type 1 CCAM/CPAM and bronchioloalveolar carcinoma suggests that type 1 CCAM/CPAM may predispose to malignant transformation by as yet unidentified tumorigenic mechanisms. Here we studied epidermal growth factor receptor (EGFR) and K-RAS oncogene, 2 biological markers closely associated with tumorigenesis and altered in many types of tumors, including lung carcinomas. For this purpose, we used immunohistochemistry and gene sequencing in paraffin-embedded tissue. Our results demonstrate expression of EGFR in types 1 and 3 CCAM/CPAM, with a distinctive distribution and intensity, compared with that of type 2. Of special interest, mucinous areas in 2 cases of type 1 CCAM/CPAM lacked EGFR expression, whereas adjacent epithelial cystic linings were strongly positive. This supports the hypothesis that mucinous differentiation in CCAM/CPAM, always present in cases with malignant transformation, could be related to other molecular pathways. The K-RAS gene was screened for mutations usually found in lung carcinomas; however, no mutations were present in any of the studied samples. These findings support the notion that EGFR may play an important role in the pathogenesis and phenotype of CCAM/CPAM.

Original languageEnglish (US)
Pages (from-to)1772-1778
Number of pages7
JournalHuman Pathology
Volume38
Issue number12
DOIs
StatePublished - Dec 2007
Externally publishedYes

Keywords

  • Bronchioloalveolar carcinoma (BAC)
  • Congenital cystic airway malformation/congenital pulmonary airway malformation (CCAM/CPAM)
  • Epidermal growth factor receptor (EGFR)
  • K-RAS

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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    Guo, H., Cajaiba, M. M., Borys, D., Gutierrez, M. C., Yee, H., Drut, R. M., Drut, R., Askin, F., Reyes-Múgica, M., & Greco, M. A. (2007). Expression of epidermal growth factor receptor, but not K-RAS mutations, is present in congenital cystic airway malformation/congenital pulmonary airway malformation. Human Pathology, 38(12), 1772-1778. https://doi.org/10.1016/j.humpath.2007.04.009