SESSION TYPE: Cancer Cases III
PRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM
INTRODUCTION: Congenital pulmonary airway malformation (CPAM) is a rare developmental anomaly of the lower respiratory tract. CPAM originates at different levels of the tracheobronchial tree and is a defect in the development of the terminal bronchioles. A hamartomatous proliferation of cysts occurs and resembles bronchioles. CPAM accounts for 25% of all congenital lung malformations. Affected patients may present with respiratory distress in the newborn period or remain asymptomatic until later in life. Most CPAMs are diagnosed and managed within the first two years of life. In rare instances such lesions may remain unrecognized until adulthood. Although frequently complicated by recurrent infection, adult CPAMs may be asymptomatic. Hence, they may be detected only incidentally on routine CXR or during a study of a non-pulmonary lesion. Malignancies in congenital CPAM are rare with a reported incidence of less than 1%.
CASE PRESENTATION: A 43 year-old female, non-smoker with no significant medical history except for 3 prior episodes of pneumonia, presented with a productive cough for 8 months. She denied fevers, chills, weight loss or constitutional symptoms. She works as general manager for a product design company and has travelled within the U.S and around the world. A chest radiograph revealed a large lucency posteriorly in the left hemithorax with an air-fluid level. A CT scan of chest confirmed a complex, irregular, thin-walled cystic structure with an air-fluid level in the left lower lobe with a communicating bronchus to the cyst. There was no separate blood supply making sequestration unlikely. Given her age and non-smoking status a beingn etiology like infected bronchocele or infected congenital cyst was entertained. She underwent an extensive work-up including fungal serologies, immune deficiency testing, complement levels, and nitoblue tetrazolidium test to evaluate neutrophil function which were negative. Cultures were all negative via bronchoscopy Due to her symptoms, she proceeded with a Robotic VATS-guided lobectomy of the left lower lobe with resection of left hilar and mediastinal lymph nodes. Pathology was positive for CPAM with invasive mucinous adenocarcinoma and metastasis identified in two of the three hilar lymph nodes.
DISCUSSION: Bronchioloalveolar carcinoma (BAC) arising in type 1 CPAM was first described in 1953 and only 16 cases have been reported since then. BAC developing within areas of CPAM are very rare and have a mean age of 26 yrs, all having mucinous characteristics. There is risk of malignant transformation in long standing CPAM which warrants close follow-up and early surgical resection is therefore recommended.
CONCLUSIONS: We report a rare case of a young female with CPAM undetected till adulthood with malignant transformation in an otherwise benign lesion.
1) MacSweeney F, et al. Am J Surg Pathol 2003; 27:1139-46
2) Stacher E, et al. Hum Pathol 2004; 35:565-70
3) Mani H, Shilo K, Histopathology 2007; 51:561-5
DISCLOSURE: The following authors have nothing to disclose: Lavanya Irugulapati, Sonali Sethi, Suhail Raoof
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