Large congenital pulmonary airway malformation with mucinous cell clusters – a case report
We report the clinical case of a 38 weeks gestational age neonate, antenatally diagnosed with a left large macrocystic pulmonary malformation conditioning dextrocardia. At birth, he presented with respiratory distress requiring non-invasive ventilation with high-flow nasal cannula (HFNC). A left inferior lobectomy was performed via thoracotomy on day 21 of life. Histological features of the lesion were compatible with congenital pulmonary airway malformation (CPAM) type I with muci- nous cell clusters. No surgical complications were reported and the neonate was discharged six days after surgery. Follow-up two months after surgery was unremarkable.
Prevalence charts and tables | EU RD Platform. https://eu-rd-platform.jrc.ec.europa.eu/eurocat/eurocat-data/ prevalence_en.
Chang, W. C. et al. Mucinous adenocarcinoma arising in congenital pulmonary airway malformation: clinicopathological analysis of 37 cases. Histopathology 2020, doi:10.1111/his.14239.
MacSweeney, F. et al. An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation. Am. J. Surg. Pathol. 2003, 27, 1139–1146.
Ota, H., Langston, C., Honda, T., Katsuyama, T. & Genta, R. M. Histochemical analysis of mucous cells of congenital adenomatoid malformation of the lung: Insights into the carcinogenesis of pulmonary adenocarcinoma expressing gastric mucins. Am. J. Clin. Pathol. 1998, 110, 450–455.
Nelson, N. D., Litzky, L. A., Peranteau, W. H. & Pogoriler, J. Mucinous Cell Clusters in Infantile Congenital Pulmonary Airway Malformations Mimic Adult Mucinous Adenocarcinoma but Are Not Associated with Poor Outcomes When Appropriately Resected. Am. J. Surg. Pathol. 2020, 44, 1118–1129.
Laje, P. & Liechty, K. W. Postnatal management and outcome of prenatally diagnosed lung lesions. Prenat. Diagn. 2008, 28, 612–618.
Durell, J. & Lakhoo, K. Congenital cystic lesions of the lung. Early Hum. Dev. 2014, 90, 935–939.
Hall, N. J. & Stanton, M. P. Long-term outcomes of congenital lung malformations. Semin. Pediatr. Surg. 2017, 26, 311–316.
Keijzer, R., Chiu, P. P. L., Ratjen, F. & Langer, J. C. Pulmonary function after early vs late lobectomy during childhood: a preliminary study. J. Pediatr. Surg. 2009, 44, 893–895.
Rittié, J. L., Morelle, K., Micheau, P., Rancé, F. & Brémont, F. Long-term outcome of bronchopulmonary malformation in children. Arch. Pediatr. 2004, 11, 520–521.
How to Cite
Copyright (c) 2022 Portuguese Journal of Cardiac Thoracic and Vascular Surgery
This work is licensed under a Creative Commons Attribution 4.0 International License.