AIMS: Congenital pulmonary airway malformation (CPAM) is a rare developmental anomaly of the lung. Here, we report a case of mucinous adenocarcinoma arising in CPAM. A 23-month-old boy underwent a thoracoscopic lobectomy of the left upper lobe of the lung based on a presumptive diagnosis of asymptomatic CPAM, found in antenatal sonogram. METHODS AND RESULTS: Histologically, the lesion was consistent with CPAM, Stocker type I. In addition, multiple foci ranging from mucinous epithelial hyperplasia to mucinous adenocarcinoma were detected. All lesions shared the same immunoprofile with the expression of cytokeratin (CK) 20, MUC5AC, and human epidermal growth factor receptor2 (HER2), but were negative for CK7, transcription factor 1 (TTF-1), MUC1, CDX2, BRAF (VE1) and anaplastic lymphoma kinase (ALK). K-RAS point mutation (G12V) was also detected in all micro-dissected mucinous lesions but EGFR mutation was not found. All lesions were consistent with mucinous adenocarcinoma. The patient's clinical course has been uneventful during the 12-months follow-up period. CONCLUSIONS: This interesting case demonstrated that multiple foci in CPAM can synchronously transform into malignancies.
AIMS: Congenital pulmonary airway malformation (CPAM) is a rare developmental anomaly of the lung. Here, we report a case of mucinous adenocarcinoma arising in CPAM. A 23-month-old boy underwent a thoracoscopic lobectomy of the left upper lobe of the lung based on a presumptive diagnosis of asymptomatic CPAM, found in antenatal sonogram. METHODS AND RESULTS: Histologically, the lesion was consistent with CPAM, Stocker type I. In addition, multiple foci ranging from mucinous epithelial hyperplasia to mucinous adenocarcinoma were detected. All lesions shared the same immunoprofile with the expression of cytokeratin (CK) 20, MUC5AC, and humanepidermal growth factor receptor2 (HER2), but were negative for CK7, transcription factor 1 (TTF-1), MUC1, CDX2, BRAF (VE1) and anaplastic lymphoma kinase (ALK). K-RAS point mutation (G12V) was also detected in all micro-dissected mucinous lesions but EGFR mutation was not found. All lesions were consistent with mucinous adenocarcinoma. The patient's clinical course has been uneventful during the 12-months follow-up period. CONCLUSIONS: This interesting case demonstrated that multiple foci in CPAM can synchronously transform into malignancies.
Authors: Jennifer Pogoriler; Daniel Swarr; Portia Kreiger; N Scott Adzick; William Peranteau Journal: Am J Surg Pathol Date: 2019-01 Impact factor: 6.394
Authors: Jacob Shujui Hsu; Ruizhong Zhang; Fanny Yeung; Clara S M Tang; John K L Wong; Man-Ting So; Huimin Xia; Pak Sham; Paul K Tam; Miaoxin Li; Kenneth K Y Wong; Maria-Mercè Garcia-Barcelo Journal: ERJ Open Res Date: 2019-02-04
Authors: Juneyoug Koh; Euiseok Jung; Se Jin Jang; Dong Kwan Kim; Byong Sop Lee; Ki-Soo Kim; Ellen Ai-Rhan Kim Journal: Korean J Pediatr Date: 2018-01-22