Congenital pulmonary airway malformation presenting as a solid mass: a description of two cases and a literature analysis
Letter to the Editor

Congenital pulmonary airway malformation presenting as a solid mass: a description of two cases and a literature analysis

Hongjin Li1,2, Lu You1,3, Qin Lou1,3, Bo Wang1,2, Rongrong Hu1,2, Xianli Luo3, Heng Liu3, Rongpin Wang2

1Graduate School of Zunyi Medical University, Zunyi, China; 2Department of Radiology, Guizhou Provincial People’s Hospital, Guiyang, China; 3Department of Radiology, Affiliated Hospital of Zunyi Medical University, Zunyi, China

Correspondence to: Rongpin Wang, MD. Department of Radiology, Guizhou Provincial People’s Hospital, No. 83, Zhongshan East Road, Nanming District, Guiyang 550002, China. Email: wangrongpin@126.com; Heng Liu, MD. Department of Radiology, Affiliated Hospital of Zunyi Medical University, No. 149, Dalian Road, Zunyi 563000, China. Email: zmcliuh@126.com.

Submitted Jun 16, 2024. Accepted for publication Oct 18, 2024. Published online Nov 14, 2024.

doi: 10.21037/qims-24-1208


Introduction

Congenital pulmonary airway malformation (CPAM) is a rare dysplasia of the lower respiratory tract, and the most common congenital pulmonary malformation. CPAM is a hamartoma-like lesion characterized by excessive hyperplasia and dilatation of the terminal bronchioles (1). CPAM is more common in newborns and infants than adults (2,3). It can occur in any lobe of both lungs, but is especially common in the lower lobes, often occurs in a single-lobe lung, and rarely occurs in two or more lobes (4-6).

Most CPAM patients have no clinical symptoms, but a few may have non-specific clinical symptoms, such as fetal edema and polyhydramnios, unexplained respiratory distress in newborns, and recurrent pulmonary infections, and hemoptysis in children and adults (7,8). Most cases of CPAM are diagnosed by prenatal ultrasound or postpartum chest radiography or computed tomography (CT) due to physical examination or other abnormalities. The examination methods commonly used to diagnose CPAM include prenatal ultrasound, magnetic resonance imaging (MRI), postnatal radiography, and CT. The imaging manifestations of CPAM are mostly single or multilocular cysts or honeycomb structures in the lungs. CPAM presenting as a solid mass is rare, difficult to diagnose, and often misdiagnosed as a lung tumor. CT examination is the most important method for the postpartum diagnosis of CPAM, and it is also an important basis for the development of treatment plans (9,10). To improve understanding and diagnosis, this article reports two cases of rare CT findings of CPAM confirmed by surgery and pathology.


Case presentation

All the procedures in this study were performed in accordance with the ethical standards of the institutional and/or national research committee(s), and the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient in Case 1 and the legal guardians of the underage patient in Case 2 for the publication of this article and any accompanying images. Copies of the written consent forms are available for review by the editorial office of this journal.

Case 1

A 27-year-old man was admitted to the Tuberculosis Department of the Affiliated Hospital of ZunyMedical University due to tuberculosis for about 5 years. The patient had previously been admitted to a local hospital following a car accident some 5 years ago. Chest CT revealed “pulmonary tuberculosis”. Anti-tuberculosis treatment was administered but the “pulmonary lesion” did not improve. During treatment, the patient experienced an occasional paroxysmal cough, no obvious expectoration, and a slight shortness of breath. The patient was then admitted to the Affiliated Hospital of Zunyi Medical University for further diagnosis and treatment. The patient had no fever, hemoptysis, chest pain, or dyspnea. The physical examination revealed coarse breath sounds in the right lung. Rhonchi, moist rale, or pleural friction rub were not heard.

Two days after admission, a chest CT plain scan and a contrast scan showed a 5.6×4.8×6.0 cm3 high-density mass in the upper lobe of the right lung, characterized by an irregular shape and slight enhancement (Figure 1A-1C). Moreover, the lower lobe of the right lung contained several cystic lesions surrounded by patchy fuzzy shadows. There was a small amount of fluid in some of the cystic lesions, and the cyst wall was thin and accompanied by calcification (Figure 1D,1E). The largest cystic lesion was about 3.3×2.8×4.5 cm3.

Figure 1 Chest CT images of a 27-year-old man with CPAM showing different manifestations of lesions on the right lung. The yellow arrows indicate the location of the lesions. (A,B) Chest CT plain scan showing a high-density mass in the upper lobe of the right lung, characterized by an irregular shape. (C) The lesion showed slight enhancement on the CT contrast scan. (D) Several cystic lesions surrounded by patchy fuzzy shadows were observed in the lower lobe of the right lung. (E) Some of the cystic lesions had a small amount of fluid. (F) Microscopically (HE, ×100), the lesion consisted of different-sized cysts, and presented with adenomatoid hyperplasia, some of which were lined with simple cuboidal epithelium. CPAM, congenital pulmonary airway malformation; CT, computed tomography; HE, hematoxylin-eosin.

The imaging diagnosis was right lung secondary pulmonary tuberculosis, and a right upper lobe mass for which a tumor diagnosis could not be excluded. Endoscopic examination showed no abnormalities in the trachea and bronchus. Laboratory tests revealed no abnormalities in the white blood cells, neutrophils, and erythrocyte sedimentation rate. The interferon gamma release assay test (peripheral blood) was negative. Multiple sputum and bronchial brushing for the tuberculosis culture, smear, and fungal culture were negative.

One week after admission, the patient underwent CT-guided percutaneous lung biopsy, and the lesion tissue was sampled, and approximately 10 mL of brown gel-like fluid was aspirated for cellular pathology, cystic fluid smear, culture, and Genxpert (Cepheid, Sunnyvale, CA, USA) examination. The results for both the tumor cells and mycobacterium tuberculosis were negative. The clinical diagnosis was a right upper lung inflammatory pseudotumor, and a right lower lung cyst accompanied by infection. The patient was transferred to the Thoracic Surgery Department for surgical treatment.

During surgery, thoracoscopic exploration revealed a right pleural adhesion, a right upper lung mass located in the apex-anterior segment, right lung horizontal fissure hypoplasia, and right middle lobe medial segment fibrosis. The right upper lung and part of the middle lobe medial segment were removed. The size of the right upper lung mass was approximately 6.0×5.0×6.0 cm3. After the mass was cut open, a brown jam-like cystic fluid was observed. The thoracoscopic exploration revealed that the mass of the right lower lung was located in the inner basal segment, near the edge of the lung, and the cyst cavity was opened and a small amount of cyst fluid was observed. The lower half of the cyst was wedge-shaped and removed by a cutting suture device. The upper half of the cyst wall was not removed from the lower lung, and the edge of the residual cyst wall was sutured. The cyst wall area was tested with water, and no air leakage was found. Microscopically, the lesions consisted of different-sized cysts and presented with adenomatoid hyperplasia, some of which were lined with simple cuboidal epithelium (Figure 1F). The pathological diagnosis was CPAM in the upper and lower lobes of the right lung.

On the eighth day after surgery, the patient was discharged without fever, chest tightness, or shortness of breath, and there was no swelling or exudation in the operation area. The patient was followed up by telephone 1 and 3 months after discharge. The patient had no fever, cough or chest discomfort, and did not follow the doctor’s advice to undergo chest radiography, CT examination, or pulmonary function examination.

Case 2

A 5-year-old female child presenting with a repeated fever and cough persisting for 2 weeks was admitted to the Affiliated Hospital of Zunyi Medical University on February 6, 2020. Two weeks earlier, the child had a fever (maximum temperature: 40 ℃), cough, and white sticky sputum. The sputum was not easy to cough out. After anti-infection treatment outside the hospital, the patient’s condition improved; the patient still had a cough, but had no expectoration, fever, or dyspnea. On physical examination, the patient had a body temperature of 36.9 ℃, the respiratory sound of the right lower lung was weakened, and no dry and wet rales or pleural friction sounds were heard. Laboratory tests revealed a white blood cell count of 18.0×109/L (neutrophils: 78%) and C-reactive protein of 29.2 mg/L. One day before admission, a chest CT scan with contrast showed a 6.8×3.6×6.5 cm3 soft tissue density mass located in the lower lobe of the right lung, characterized by well-defined margins and homogeneous density. The contrast scan showed mildly heterogeneous enhancement, and a patchy low-density area without enhancement. The mediastinal lymph nodes were enlarged (Figure 2A-2E). The imaging diagnosis was right lower lobe inflammatory lesions for which a tumor diagnosis could not be excluded.

Figure 2 A 5-year-old girl with CPAM showed manifestations of a lesion in the lower lobe of the right lung at different periods. The red arrows indicate the location of the lesion. (A-E) First chest CT scan. (F) Chest CT scan 11 days later. (G,H) Chest CT scan 2 months later. (A,B) Chest CT plain scan and contrast scan showing a soft tissue density mass in the lower lobe of the right lung, with clear edges and uniform density. (C,D) Scans showing a patchy low-density area without enhancement. (E) Scan showing enlarged mediastinal lymph nodes. (F) Chest CT showing multiple honeycomb cysts of different sizes in the dorsal and posterior basal segments of the lower lobe of the right lung with uneven wall thickness. (G) Chest CT showing multiple cysts of different sizes in the lower lobe of the right lung; some of the cyst walls were slightly thicker. (H) Chest CT showing a thin-walled cyst in the lower lobe of the right lung, with a clear boundary, surrounded by a cord-like high-density shadow. (I) Microscopically (HE, ×100), histopathology revealed cyst cavities of different sizes. The cyst wall was covered with a single layer of ciliated columnar and simple cuboidal epithelium. Smooth muscle tissue was observed in the wall. CPAM, congenital pulmonary airway malformation; CT, computed tomography; HE, hematoxylin-eosin.

The patient was then admitted for treatment. Eleven days after admission and anti-infection treatment, the patient’s condition had improved, and the patient had no fever, cough, or shortness of breath. On February 17, 2020, chest CT showed multiple honeycomb cysts of different sizes in the dorsal and posterior basal segments of the lower lobe of the right lung with uneven wall thickness (Figure 2F). The largest cystic lesion was about 4.3×2.1×4.5 cm3. The mediastinal lymph node was smaller than that on the previous CT. The imaging diagnosis was CPAM in the lower lobe of the right lung.

After 2 weeks of hospitalization, the patient was discharged. On March 12, 2020, the patient underwent chest CT examination as an outpatient, and chest CT showed multiple cysts of different sizes in the lower lobe of the right lung; some of the cyst walls were slightly thicker, surrounded by fewer lesions, and had clearer boundaries compared with those on the previous CT (Figure 2G). The mediastinal lymph nodes were smaller than those observed on the previous CT.

The patient was readmitted to hospital on May 28, 2020. On May 31, 2020, chest CT revealed a thin-walled cyst in the lower lobe of the right lung, with a clear boundary, surrounded by a small cord-like high-density shadow (Figure 2H). No enlargement of the mediastinal lymph nodes was observed. On June 2, 2020, during surgery, thoracoscopic exploration showed partial consolidation of the lower lobe of the right lung, and vesicle-like lesions of varying sizes were visible on the surface (the largest of which was about 3 cm in diameter). The boundary of the lesions was unclear and diffusely distributed in the lower lobe of the right lung. The lower lobe of the right lung partially adhered to the chest wall. The lower lobe of the right lung was removed and then cut open, and more cystic cavities were observed with a large amount of pus and obvious pulmonary edema. Histopathology revealed cystic cavities of different sizes. The cyst wall was covered with simple cuboidal epithelium, and smooth muscle tissue was observed in the wall (Figure 2I). The pathological diagnosis was CPAM in the lower lobe of the right lung.

One week after surgery, the patient was discharged without fever, cough, or sputum, and there was no redness, swelling, or exudation in the surgical area. One month after surgery, a chest radiography showed a small amount of pleural effusion and pneumothorax in the right chest cavity. Follow-up chest radiography scans 3 and 8 months after surgery showed good inflation of both lungs. The patient had no fever, cough, or chest discomfort. The entire diagnosis and treatment process of the patient is summarized in a flowchart (Figure 3).

Figure 3 Timeline of the patient’s diagnosis and treatment processes. CT, computed tomography.

Discussion

CPAM is a developmental lung malformation characterized by an abnormal airway pattern that occurs during lung branching morphogenesis, and can lead to cystic or adenomatous pulmonary areas (11). Currently, the etiology of CPAM is unclear. Generally, due to the influence of unknown factors during the development of fetal lung buds, signal transduction between epithelial and lower mesenchymal cells is blocked, resulting in proximal airway defects in the formation of bronchi and terminal bronchioles, an arrest in development at 8–16 weeks of the pseudoglandular, stage and no acinar formation in the early canalicular stage (11,12). Most children with CPAM have no obvious symptoms after birth, but a few children have severe respiratory and circulatory disorders due to the compression of the heart, lung, and mediastinum. CPAM presents a risk for secondary pulmonary infection, and certain types of CPAM have an increased risk of malignancy (13,14). If no infection occurs, surgical resection is the primary treatment for CPAM, and can effectively prevent complications (3,15). Treatment of any infection prior to surgery depends on the radiographic findings.

Based on the clinical and pathological features, Stocker divided CPAM into five types (i.e., types 0, I, II, III, and IV). Type 0 CPAM originates from the trachea and bronchial structures, and has been previously described as acinar dysplasia. Type I CPAM originates from the bronchi or bronchioles, and is characterized by one or more cysts more than 2 cm in diameter. Type II CPAM originates from the bronchioles and consist of multiple cysts 0.5–2 cm in diameter. Type III CPAM originates from the bronchioles or alveolar ducts, and mainly presents as solid masses accompanied by microcysts less than 0.5 cm in diameter. Type IV CPAM originates from the peripheral cysts of alveolar cells (16,17). Types I and II are the most common types of CPAM, and solid masses are rare in these types of CPAM.

With the development of medical imaging technology, fetal ultrasound, MRI, postnatal radiography, and CT are widely used in the diagnosis of congenital pulmonary diseases. Obtaining accurate imaging data is important in the clinical diagnosis and treatment of these diseases (15,18,19). Ultrasound and MRI are the main examinations used in the fetal period. Ultrasound examinations have the advantages of convenience, low cost, good repeatability, and no X-ray radiation (20,21). Conversely, while MRI examination has no radiation, it takes a long time and has high costs; however, a fetal MRI may be performed if CPAM is detected on antenatal ultrasound (1). Chest radiography and CT are the main examinations after birth. The diagnosis of CPAM based on chest radiography alone has great limitations, and the misdiagnosis rate is high (22). CT is of great value in localization, diagnosis, and differential diagnosis. It can be used to help determine the scope of preoperative planning and provides detailed information for treatment management (9,10,23). However, CT examination has X-ray radiation, and sedatives often need to be administered to pediatric patients before they can undergo the examination. If pediatric patients require multiple chest CT examinations for evaluation, low-dose CT scans may be appropriate, but radiation protection for the patients should be emphasized (24,25). A previous study of 103 infants who underwent surgical resection for congenital lung malformation reported that CT diagnosis had a concordance rate of 84% with the histological diagnosis of CPAM (26). Wu et al. (23) retrospectively analyzed the clinical and CT data of children with pathologically diagnosed CPAM, and found that the main CT manifestations of CPAM were cysts and pneumonia. In relation to pathological findings, the characteristics of cysts are important for the identification of cystic CPAM.

The imaging findings of CPAM mainly depend on the histological subtype and the presence or absence of infection. Based on our literature review (3,23,27,28) and cases, the imaging features of CPAM are summarized as follows: (I) one lung-lobe involvement, with a predilection for the lower lobe; it is rare that more than one lobe is involved; (II) single or multiple air-containing cysts surrounded by irregular small cystic structures, some showing air-fluid levels; (III) small cysts that present as honeycomb-like structures (mostly adjacent to the pleura) that are relatively uniform in size. When accompanied by infection, the cyst wall thickens, fluid accumulates in the cyst, and air-fluid levels may be overserved; and (IV) cystic-solid or solid masses with well-defined borders, containing irregular small cysts.

The patient in case 1 was an adult with lesions in the two lung lobes. The lesion in the right upper lobe presented as a solid mass, which is extremely rare. Due to the presence of tiny cysts and adenomat-like structures that are difficult to distinguish with the naked eye, it was misdiagnosed as a tumor before surgery. The lesion in the lower lobe of the right lung was a multicystic lesion with a thin cystic wall and calcification. It contained little visible fluid and was surrounded by patchy and spotted shadows that could be easily misdiagnosed as pulmonary tuberculosis. In case 1, the patient’s multiple sputum, bronchial brushing tuberculosis culture, and smear were negative.

In case 2, the first CT finding was a solid mass in the lower lobe of the right lung with clear boundaries, uneven enhancement, a patchy low-density area without visible enhancement, and enlarged mediastinal lymph nodes. Following the first CT scan, a lung tumor was suspected. Based on symptoms such as fever, cough, and an elevated white blood cell count, an anti-infection treatment was initiated. After 11 days of anti-infection treatment with ceftazidime, a chest CT scan showed that the solid mass in the lower lobe of the right lung had evolved into multiple cystic lesions of varying sizes and the mediastinal lymph nodes were significantly smaller than those in the previous chest CT findings. The CT scans of the lung lesions displayed typical CPAM imaging manifestations. Currently, the CT diagnosis of CPAM is easy. A key point in differentiating CPAM from a pulmonary neoplastic lesion is that a pulmonary neoplastic lesion does not shrink after the infection has been treated. Subsequently, two chest CT examinations showed that the lesions around the right lower lung cystic cavity had gradually decreased, and the boundaries of the cyst walls were clear. The first CT manifestation in case 2 was a solid mass, which was caused by both CPAM and the infection. After anti-infection treatment, the cyst contents were discharged through bronchial drainage, and presented as cystic lesions of varying sizes with typical CT features of CPAM.

Due to the typical cystic changes, CPAM diagnosis is not challenging. When a local polycystic lesion involving a single lobe (especially the lower lobe) occurs in the lungs of children, CPAM should be considered first, regardless of whether the capsule content is gas, liquid, or a gas-liquid mixture, and regardless of whether it is combined with patchy high-density shadows. However, it should be differentiated from other common congenital lung diseases with similar manifestations such as pleuropulmonary blastoma (PPB), bronchopulmonary sequestration (BPS), congenital lobar overinflation (CLO), and congenital diaphragmatic hernia (CDH).

PPB is a common pulmonary malignant tumor in children. The imaging manifestations of PPB are similar to those of CPAM, and the differential diagnosis is difficult. If chest CT shows calcification and mural nodules in the cyst wall, a diagnosis of PPB should be considered. PPB is a common tumor in DICER1 syndrome. The occurrence of PPB is related to the DICER1 gene mutation. DICER1 gene detection is helpful for the diagnosis and differential diagnosis of PPB (29,30).

BPS is composed of nonfunctioning lung tissue that is not connected to the bronchial tree. It is a solid-mass lung disease with systemic arterial blood supply that usually originates from the thoracic aorta. CT angiography or magnetic resonance angiography shows an abnormal blood supply from the systemic circulation, and this is the key point for distinguishing it from CPAM.

CLO, previously termed congenital lobar emphysema, often manifests as excessive expansion, decreased density, and sparse markings on one or more lung lobes. However, there are no cystic lesions in the low-density area of CLO, which helps to distinguish it from CPAM.

CDH manifests as a decrease in the abdominal aerated structure and an unclear ipsilateral diaphragmatic surface. If CDH is suspected, the oral administration of contrast agents can help with the diagnosis and differential diagnosis. If the CPAM image shows a solid mass, the diagnosis is difficult, and it is often misdiagnosed as a lung tumor. The diagnosis depends on pathology, immunohistochemistry and gene detection.


Conclusions

CPAM is most common in newborns and infants. It commonly occurs in a single lobe of the lung, and rarely occurs in two or more lobes of the lungs. CT examination is the most important method for the diagnosis of CPAM. CT has important value in the localization, diagnosis, and differential diagnosis of lesions, and it is also an important basis for formulating treatment plans. Notably, contrast scans enable the nature of the lesion to be further analyzed based on the degree of enhancement. However, issues, such as the dosage and allergic reactions, need to be considered in the use of contrast scans. CPAM often presents as a cystic mass with specific imaging characteristics, and can be easily diagnosed. CPAM that presents as a solid mass is rare, and its imaging diagnosis is difficult. It is often misdiagnosed as a pulmonary neoplastic lesion, and the diagnosis requires a pathological examination. Calcification and mural nodules in the cyst wall can be used to differentiate between PPB and CPAM. If the image shows a solid mass converted into cystic lesions after treatment for infection, patients should be diagnosed with CPAM rather than neoplastic lesions.


Acknowledgments

The authors would like to sincerely thank the adult patient, and the child patient and her guardians for their participation. The authors would also like to thank Editage (www.editage.com) for undertaking the English language edit.

Funding: This study was funded by the Guiyang Science and Technology Project (No. ZKHT [2022]-4-1-3), and the Science and Technology Basic Research Program Project of Guizhou Province (No. Qiankehejichu-ZK [2021] 486).


Footnote

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-24-1208/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures in this study were performed in accordance with the ethical standards of the institutional and/or national research committee(s) and the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient in case 1 and the legal guardians of the underage patient in case 2 for the publication of this article and any accompanying images. Copies of the written consent forms are available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.


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Cite this article as: Li H, You L, Lou Q, Wang B, Hu R, Luo X, Liu H, Wang R. Congenital pulmonary airway malformation presenting as a solid mass: a description of two cases and a literature analysis. Quant Imaging Med Surg 2024;14(12):9805-9812. doi: 10.21037/qims-24-1208

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