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Lung Pathology |

Pulmonary Congenital Cystic Adenomatoid Malformation Stocker Class Type 1 in a 26 Year-Old Female

Beatrice Tan, MD; Jose Paolo Panuda, MD; Consuelo Tan, MD; Albert Rafanan, MD
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Chong Hua Hospital, Cebu City, Philippines


Chest. 2015;148(4_MeetingAbstracts):624A. doi:10.1378/chest.2270284
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Abstract

SESSION TITLE: Lung Pathology Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Congenital Cystic Adenomatoid Malformation (CCAM) of the lung is an uncommon fetal developmental anomaly of the terminal respiratory structures. Almost all cases are diagnosed during the first two years of life. Its presentation in adulthood is extremely rare, with only 27 cases world-wide(1).

CASE PRESENTATION: This is a case of a 26 year-old female presenting as recurrent flight-related dyspnea that spontaneously resolved after landing. In one of her check-up, chest radiograph showed a large cystic mass with air-fluid level in the right upper lobe (Figure 1). Chest CT scan with contrast revealed an 8.7 x 7.9 x 13.6 cm pneumatocele in the apical and posterior segments of the right upper lobe with areas of septation and air-fluid level suggestive of an infected cyst/pneumatocele. Patient underwent lobectomy by open thoracotomy. A huge bullae occupying 95% of the right upper lobe approximately 15 cm in its widest diameter was seen, with loculations filled with brownish fluid. The inner surface of the cyst had irregular interlacing surface and fibrous strands (Figure 2). Histopathologic examination of the cyst revealed multiple cystic masses lined by ciliated pseudostratified columnar epithelium consistent with CCAM type 1. Patient had unremarkable post-operative course and was discharged improved.

DISCUSSION: Pre-operative diagnosis of adult CCAM is difficult because of variable clinical and radiologic expressions. The most common presentation of adult CCAM is productive cough and hemoptysis. Other symptoms such as fever, recurrent infection and dyspnea secondary to pneumothorax occur less often. In this case, our patient had a unique presentation of flight-associated dyspnea that has not been reported previously. CT scan of the chest is the optimal diagnostic modality as it helps delineate the anatomic extent of the anomalies for surgical planning. There are three classifications of CCAM according to Stoker. Type 1 consists of single or multiple large cysts more than 2 cm in diameter and is lined by ciliated pseudostratified columnar epithelium with normal alveoli seen between cysts. Type 1 CCAM carries a better prognosis compared to type 2 and 3 but is associated with development of mucinous bronchioalveolar carcinoma. Treatment is by complete pulmonary cyst resection by lobectomy either by video assisted thoracic surgery or by conventional open thoracotomy. In this case, open lobectomy was done successfully with no post-operative complications.

CONCLUSIONS: We presented a 26 year-old female with Stocker Type 1 CCAM who presented with recurrent flight-related dyspnea, underwent lobectomy by open thoracotomy, without noted recurrence post-operatively.

Reference #1: Herrero Y, Pinilla I, Torres I, et al. Cystic adenomatoid malformation of the lung presenting in adulthood. Ann of Thoracic Surg. 2005; 79:326-329.

DISCLOSURE: The following authors have nothing to disclose: Beatrice Tan, Jose Paolo Panuda, Consuelo Tan, Albert Rafanan

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