Cystic adenomatoid malformation in adults: radiological findings and pathologic correlation

Transcription

1 Cystic adenomatoid malformation in adults: radiological findings and pathologic correlation Poster No.: C-1734 Congress: ECR 2014 Type: Educational Exhibit Authors: C. Batz Colvée, M. Vidal, M. Ruiz Tolón, A. M. Gómez Martínez, N. T. Escribano Adam, I. de la Pedraja; Madrid/ES Keywords: Congenital, Diagnostic procedure, MR, CT, Conventional radiography, Thorax, Lung DOI: /ecr2014/C-1734 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 29

2 Learning objectives - To describe the radiological findings in 12 adult cases of congenital cystic adenomatoid malformation (CCAM) diagnosed in our institution within the past 14 years, including their pathologic correlation. - To compare these findings with those in literature and to describe other entities that should be included in the differential diagnosis. Page 2 of 29

3 Background Congenital Cystic Adenomatoid Malformation (CCAM) is an heterogeneous group of cystic and non-cystic lung lesions caused by an early airway maldevelopment, being Congenital Pulmonary Airway Malformation (CPAM) a preferable term to encompass them. It represents 25% of all pulmonary congenital lesions, usually manifested as recurrent pneumonias during childhood although it might be incidentally diagnosed in adults. The lesion consists of adenomatoid proliferation of bronchioles that form cysts instead of normal alveoli due to a stop in the acinar phase of pulmonary maturation, frequently unilobar. They have an abnormal communication with the airway that permits the air path so the lesions have a variable amount of air and fluid inside. Therefore, their typical appearance in imaging is that of cystic lesions with air-fluid level inside that may change over time. They usually get the blood supply from the pulmonary artery and drain via the pulmonary veins, however an abnormal systemic arterial supply can be seen in CPAMs, referred as hybrid lesions and demonstrating imaging features of both CPAM and bronchopulmonary sequestration. It was first described by Ch in and Tang in 1949 and Stocker made the actual classification in 2002, including five different types regarding to the size of the cysts and their histological characteristics. Type 0 has a tracheal or bronchial origin and is really acinar dysgenesis or dysplasia. Type 1 has a bronchial or bronchiolar origin, the most frequent one (60-70%): cystic lesions > 2cm lined by bronchial-type epithelium with a small amount of supporting fibrous tissue and smooth muscle, but no cartilage. Type2 has a bronchiolar origin: cystic lesions < 2cm lined by bronchial-type epithelium. Type 3 has a bronchiolar - alveolar duct origin: adenomatoid type. Alveolar duct-like structures lined by low cuboidal epithelium without normal lung airspaces. Occasionally small cysts (<0,5 cm) may be present. Page 3 of 29

4 Type 4 has a distal acinar origin: the "unlined cyst lesion", appearing as a large cyst indistinguishable from a predominantly cystic pleuropulmonary blastoma by imaging. Page 4 of 29

5 Findings and procedure details We have compiled 12 (8 women and 4 men) cases of histologically proven CAM, diagnosed in adults during the past 14 years at our institution. We have retrospectively reviewed the radiological findings in different imaging techniques being the conventional radiography the common method for a first diagnostic approach in these patients. The most common appearance in imaging was a predominantly multicystic lesion with a variable amount of air-fluid level inside (Figs 1 and 2). The location of the lesion was found to be equal in both right and left lower lobes (4 patients in each hemithorax). 3 patients had the superior right lobe involved and 1 the superior left lobe. All patients were disgnosed with CAM type 1 and the ages at the time of diagnosis were comprised between years old. Only one patient had 14 years old at the time of diagnosis, which was the only case of CAM type 2. The most frequent clinical manifestation was hemoptysis (5 / 12 patients) followed by pneumonia (4 / 12 patients). 2 patients consulted for dyspnea and 1 presented as acute pneumothorax (Figs 16, 17 and 18). The most important differential diagnosis is with malignancy. We show two cases of MAQ that appeared as neoplastic pathology (Figs 10-15) and went through extensive surgery because of this misinterpretation. Pulmonary carcinoma and cystic pleuropulmonary blastoma are the two malignant entities that shold be taken into consideration. Evolution of the lesions and patient age can help us to distinguish this pathologies, but only the histological study is definitive. The benign pathologies included in the differential diagnosis are mainly bronchogenic cyst, cavitary necrosis complicating pneumonia, congenital lobar overinflation, cystic limphangioma, bronchial atresia and bronchiectasias. Pulmonary sequestration may be associate with CAM and those cases are refered as hybrid lesions. Page 5 of 29

6 Images for this section: Fig. 1: AP and L chest radiography showing the most frequent appearance of MAQ: Cystic intrapulmonary well-circumscribed lesion in the lower lobes (mostly in the right lung) with air-fluid level inside. Page 6 of 29

7 Fig. 2: AP and L chest radiography showing the most frequent appearance of MAQ: Cystic intrapulmonary well-circumscribed lesion in the lower lobes (mostly in the right lung) with air-fluid level inside. Page 7 of 29

8 Fig. 3: PA chest radiography demonstrating a cystic lesion with multiple thin septa in the lower left lobe. Page 8 of 29

9 Fig. 4: Thorax enhanced CT: same lesion as Fig.3. Well defined multiple cystic mass with thin walls inside, located in the lower left lobe. No solid areas or air-fluid levels are seen. Page 9 of 29

10 Fig. 5: Gross specimen revealing multiple cystic spaces. Page 10 of 29

11 Fig. 6: Cystic intrapulmonary spaces of varying size, lined by cylindrical epithelium. There is cartilage within the cystic wall. Page 11 of 29

12 Fig. 7: Postcontrast CT: Multicystic lesion in the upper right lobe. Note the airway communication. Page 12 of 29

13 Fig. 8: Sagital plane demonstrating the airway communication between the lesion and the bronchus. Page 13 of 29

14 Fig. 9: Multiple cystic intrapulmonary spaces lined by cylindrical epithelium. The fibrous wall has small foci of smooth muscle and vascular structures. Page 14 of 29

15 Fig. 10: PA chest radiography: solid lesion in the lower left lobe. No cystic images or airfluid level are seen. Page 15 of 29

16 Fig. 11: PET-CT of the same patient showing a predominantly solid lesion in the lower left lobe. Page 16 of 29

17 Fig. 12: Fusion PET-CT image illustrating no FDG metabolism. Page 17 of 29

18 Fig. 13: Chest x-ray showing a small lesion with both cystic and solid characteristics, in the upper right lobe. Page 18 of 29

19 Fig. 14: Non enhaced thorax CT demonstrating the predominantly cystic lesion. It has a solid and irregular zone that suggested malignancy. Page 19 of 29

20 Fig. 15: Large gross specimen of a cystic pulmonary lesion with no neoplastic growth. Page 20 of 29

21 Fig. 16: This patient presented with an accute pneumothorax, collapsed right lung and mediastinal shift. Notice the cystic well-circumscribed lesion located in the lower right hemithorax. Page 21 of 29

22 Fig. 17: This patient presented with an accute pneumothorax, collapsed right lung and mediastinal shift. Notice the cystic well-circumscribed lesion located in the lower right hemithorax. Page 22 of 29

23 Fig. 18: A reformatted image that shows the original position of the big cystic lesion, once the pneumothorax was resolved. Page 23 of 29

24 Fig. 19: MR Axial FIESTA image showing a cystic lesion with an air-fluid level in the lower right lobe. Page 24 of 29

25 Fig. 21: Sagital 3D sequence of the same finding. Page 25 of 29

26 Fig. 20: Postcontrast sagital MR image demonstrating no enhacement of the lesion. Page 26 of 29

27 Conclusion Although Cystic Adenomatoid Malformation is an entity usually disgnosed in the pediatric age group it is important for the general radiologists to keep in mind this pathology as it can be detected in adulthood and some times misinterpreted as malignancy. Page 27 of 29

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