Impact of combined pulmonary fibrosis and emphysema on lung cancer risk and mortality in rheumatoid arthritis: A multicenter retrospective cohort study.

OBJECTIVE: Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome characterized by the coexistence of emphysema and fibrotic interstitial lung disease (ILD). The aim of this study was to examine the effect of CPFE on lung cancer risk and lung cancer-related mortality in patients with rheumatoid arthritis (RA). METHODS: We conducted a multicenter retrospective cohort study of patients newly diagnosed with lung cancer at five community hospitals between June 2006 and December 2021. Patients were followed until lung cancer-related death, other-cause death, loss to follow-up, or the end of the study. We used the cumulative incidence function with Gray's test and Fine-Gray regression analysis for survival analysis. RESULTS: A total of 563 patients with biopsy-proven lung cancer were included (82 RA patients and 481 non-RA patients). The prevalence of CPFE was higher in RA patients than in non-RA patients (40.2% vs.10.0%) at lung cancer diagnosis. During follow-up, the crude incidence rate of lung cancer-related death was 0.29 and 0.10 per patient-year (PY) in RA and non-RA patients, and 0.32 and 0.07 per PY in patients with CPFE and patients without ILD or emphysema, respectively. The estimated death probability at 5 years differed between RA and non-RA patients (66% vs. 32%, p<0.001) and between patients with CPFE and patients without ILD or emphysema (71% vs. 24%, p<0.001). In addition to clinical cancer stage and no surgery within 1 month, RA and CPFE were identified as independent predictive factors for increased lung cancer-related mortality (RA: adjusted hazard ratio [HR], 2.49; 95% confidence interval [CI], 1.65-4.76; CPFE: adjusted HR 2.01; 95% CI 1.24-3.23). CONCLUSIONS: RA patients with lung cancer had a higher prevalence of CPFE and increased cancer-related mortality compared with non-RA patients. Close monitoring and optimal treatment strategies tailored to RA patients with CPFE are important to improve the poor prognosis of lung cancer.

Nine controversial questions about augmentation therapy for alpha-1 antitrypsin deficiency: a viewpoint.

Augmentation therapy with intravenous alpha-1 antitrypsin is the only specific treatment for alpha-1 antitrypsin deficiency (AATD)-associated emphysema. This treatment has been available and remained basically unchanged for more than 35 years, but many questions persist regarding its indications, regimen of administration and efficacy. Because AATD is a rare disease, it has not been possible to conduct randomised, placebo-controlled trials that are adequately powered for the usual outcomes analysed in non-AATD-related COPD, such as lung function decline, exacerbations, symptoms or quality of life. New outcomes such as lung densitometry measured by computed tomography are more sensitive for identifying emphysema progression but are not widely accepted by regulatory agencies. In addition, clinical manifestations, severity and the natural history of lung disease associated with AATD are very heterogeneous, which means that individual prediction of prognosis is challenging. Therefore, the indication for augmentation is sometimes a dilemma between initiating treatment in individuals who may not develop significant lung disease or in whom disease will not progress and delaying it in patients who will otherwise rapidly and irreversibly progress.Other areas of debate are the possible indication for augmentation in patients with severe AATD and respiratory diseases other than emphysema, such as bronchiectasis or asthma, and the use of therapy after lung transplant in AATD patients. All these uncertainties imply that the indication for treatment must be personalised in expert reference centres after in-depth discussion of the pros and cons of augmentation with the patient.

Surgical and bronchoscopic pulmonary function-improving procedures in lung emphysema.

COPD is a highly prevalent, chronic and irreversible obstructive airway disease without curative treatment. Standard therapeutic strategies, both non-pharmacological and pharmacological, have only limited effects on lung function parameters of patients with severe disease. Despite optimal pharmacological treatment, many patients with severe COPD still have a high burden of dyspnoea and a poor quality of life. If these patients have severe lung emphysema, with hyperinflation as the driver of symptoms and exercise intolerance, lung volume reduction may be an effective treatment with a significant impact on lung function, exercise capacity and quality of life. Currently, different lung volume reduction approaches, both surgical and bronchoscopic, have shown encouraging results and have been implemented in COPD treatment recommendations. Nevertheless, choosing the optimal lung volume reduction strategy for an individual patient remains challenging. Moreover, there is still room for improving durability of effect and safety in all available procedures. Ongoing and innovative research is essential to push this field forwards. This review provides an overview of results and limitations of the current lung volume reduction options for patients with severe lung emphysema and hyperinflation.

Combined Pulmonary Fibrosis and Emphysema: A Narrative Review.

Combined pulmonary fibrosis and emphysema (CPFE) syndrome refers to co-occurrence of two disease processes in the lung that can be difficult to diagnose but is associated with high morbidity and mortality burden. Diagnosis of CPFE is challenging because the two diseases can counterbalance respective impairments resulting in deceivingly normal-appearing chest radiography and spirometry in a dyspneic patient. Although an international committee published the terminology and definitions of CPFE in 2022, consensus on exact diagnostic criteria and optimal management strategy is yet to be determined. Herein, we provide a narrative review summarizing the literature on CPFE from 1990 to 2022, including historical background, epidemiology, pathogenesis, clinical features, imaging and pulmonary function findings, diagnosis, prognosis, complications, and treatment. Although CPFE was initially conceived as a variant presentation of idiopathic pulmonary fibrosis, it has been recognized to occur in patients with a wide variety of interstitial lung diseases, including connective tissue disease-associated interstitial lung diseases, and hypersensitivity pneumonitis. The affected patients have a heightened risk for pulmonary hypertension and lung cancer. Clinicians need to recognize the characteristic presenting features of CPFE along with prognostic implications of this entity.

Pathogenesis and management of emphysema in people with HIV.

INTRODUCTION: Since early in the HIV epidemic, emphysema has been identified among people with HIV (PWH) and has been associated with increased mortality. Smoking cessation is key to risk reduction. Health maintenance for PWH and emphysema should ensure appropriate vaccination and lung cancer screening. Treatment should adhere to inhaler guidelines for the general population, but inhaled corticosteroid (ICS) should be used with caution. Frontiers in treatment include targeted therapeutics. Major knowledge gaps exist in the epidemiology of and optimal care for PWH and emphysema, particularly in low and middle-income countries (LMIC). AREAS COVERED: Topics addressed include risk factors, pathogenesis, current treatment and prevention strategies, and frontiers in research. EXPERT OPINION: There are limited data on the epidemiology of emphysema in LMIC, where more than 90% of deaths from COPD occur and where the morbidity of HIV is most heavily concentrated. The population of PWH is aging, and age-related co-morbidities such as emphysema will only increase in salience. Over the next 5 years, the authors anticipate novel trials of targeted therapy for emphysema specific to PWH, and we anticipate a growing body of evidence to inform optimal clinical care for lung health among PWH in LMIC.

[Treatment of chronic obstructive pulmonary disease and emphysema]. / Metody lecheniya khronicheskoi obstruktivnoi bolezni i emfizemy legkikh.

We summarized the available data on therapeutic, surgical and endoscopic treatment of chronic obstructive pulmonary disease and emphysema that may be used like a bridge to lung transplantation. Treatment of chronic obstructive pulmonary disease and emphysema is expensive. Certain limitations in lung transplantation make to create new methods of treatment of severe emphysema. However, one should be ready for possible complications and carefully select patients for certain treatment to avoid false negative results. Reducing costs or developing cheaper treatments is important for the future and availability of care. The risks and complications associated with surgical treatment of emphysema can make endoscopic surgery preferable for these patients, and this undoubtedly requires further research.

Endobronchial Valve Replacements in Patients with Advanced Emphysema After Endoscopic Lung Volume Reduction.

Purpose: Up to 41% of patients with endobronchial valve implantation need revision bronchoscopies and valve replacements most likely due to valve dysfunction or lack of benefit. So far, no data is available whether valve replacements lead to the desired lobar volume reduction and therapy benefit. Patients and Methods: We conducted a single-center retrospective analysis of patients with endobronchial valve implantation and at least one valve replacement. Indications and number of revision bronchoscopies and valve replacements were evaluated. Therapy benefit regarding lung function and exercise capacity as well as development of complete lobar atelectasis was investigated and possible predictors identified. Results: We identified 73 patients with 1-12 revision bronchoscopies and 1-5 valve replacements. The main indication for revision bronchoscopy in this group was lack of therapy benefit (44.2%). Lung function and exercise capacity showed improvements in about one-third of patients even years after the initial implantation. A total of 26% of all patients showed a complete lobar atelectasis at the end of the observation period, 56.2% had developed lung volume reduction. The logistic regression revealed the development of a previous complete lobar atelectasis as predictor for a complete lobar atelectasis at final follow-up. Oral cortisone long-term therapy was also shown as predictive factor. The probability for a final complete lobar atelectasis was 69.2% if a lobar atelectasis had developed before. Conclusion: Valve replacements are more likely to be beneficial in patients who develop a re-aeration of a previous lobar atelectasis following valve implantation. Every decision for revision bronchoscopy must be taken carefully.

[Bronchoscopic lung volume reduction]. / Endoskopische Lungenvolumenreduktion.

Bronchoscopic lung volume reduction is as a safe and effective therapy for patients with advanced emphysema, suffering from breathlessness despite optimal medical therapy. By reducing hyperinflation, it improves lung function, exercise capacity and quality of life. The technique includes one-way endobronchial valves, thermal vapor ablation and endobronchial coils. Patient selection is the key to a successful therapy; hence the indication should be evaluated in a multidisciplinary emphysema team meeting. The procedure can lead to a potentially life-threatening complication. Therefore, an adequate post-procedural patient management is crucial.

Lung transplantation for COPD/pulmonary emphysema.

COPD and α-1 antitrypsin deficiency emphysema remain one of the major indications for lung transplantation. If all other treatment possibilities are exhausted or not possible (including rehabilitation, oxygen therapy, noninvasive ventilation, lung volume reduction), patients may qualify for lung transplantation. Strict selection criteria are implemented with a lot of relative and absolute contraindications. Because of an ongoing donor shortage, only a minority of endstage COPD patients will finally get transplanted. The procedure may involve a single or a double lung transplantation, dependent on the experience of the centre, the waiting list, the availability of donor lungs and the patient's risk-benefit ratio. In general, the life expectancy as well as the health-related quality of life after lung transplantation for COPD are usually increased, and may be somewhat better after double compared with single lung transplantation. Several specific complications can be encountered, such as the development of solid organ cancer and chronic lung allograft dysfunction, which develops in up to 50% of patients within 5 years of their transplant and has a major impact on long-term survival, because of the current inefficient treatment modalities.

Impact of Interstitial Lung Abnormalities on Disease Expression and Outcomes in COPD or Emphysema: A Systematic Review.

Background: Both COPD and interstitial lung abnormalities (ILAs) are conditions associated with smoking and age. The impact of coexistent ILAs on the manifestations and outcomes of COPD or emphysema awaits evaluation. Methods: We searched PubMed and Embase using Medical Subject Headings terms in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Results: Eleven studies were included in the review. The sample size of the studies ranged from 30 to 9579. ILAs were reported in 6.5% to 25.7% of the patients with COPD/emphysema, higher than that reported in the general populations. COPD/emphysema patients with ILAs were older, mostly male, and had a higher smoking index than those without ILAs. Hospital admission and mortality were increased in COPD patients with ILAs compared to those without ILAs, whereas the frequency of COPD exacerbations was discrepant in 2 of the studies. The FEV1 and FEV1% predicted tended to be higher in the group with ILAs, but not significantly in most of the studies. Conclusion: ILAs were more frequent in subjects with COPD/emphysema than in the general population. ILAs may have a negative impact on hospital admission and mortality of COPD/emphysema. The impact of ILAs on lung functions and exacerbations of COPD/emphysema was discrepant in these studies. Further prospective studies are warranted to provide high-quality evidence of the association and interaction between COPD/emphysema and ILAs.

The long-term outcomes of symptomatic congenital lobar emphysema patients.

INTRODUCTION: Surgical (OP) management for symptomatic congenital lobar emphysema (CLE) is the standard of care with nonoperative (NOP) approach applied for asymptomatic cases. The aim of this study is to report the outcomes for NOP approach to the care of symptomatic CLE infants. METHODS: A retrospective study of CLE patients treated 2000-2021 at a single institution. Patients with CLE and respiratory symptoms were included. RESULTS: Overall, 23 children had symptomatic CLE, and 12 had NOP management. The median age at diagnosis was 38.5 days (50) in the NOP group versus 25 days (20) in the OP group (p = 0.31). There was no significant difference in the location of the involved lobe, term birth, postnatal diagnosis and gender, and both groups required noninvasive support in 33% of the cases. There was a trend towards higher frequency of oxygen support in the OP group preoperatively (89% vs. 42%, p = 0.07). The median length of stay was 14 days in the NOP group compared to a median postsurgery stay of 7.5 days in the OP group. In follow-up, there was no significant difference in respiratory readmission in first year of life, growth delay, treatment with asthma medication or body mass index in the NOP versus OP group. None of the children in the NOP group required surgery during follow-up. CONCLUSIONS: A NOP approach for symptomatic CLE infants can have favorable long-term outcomes. Further studies will be required to identify markers to aid in clinical decision-making.

Outcomes of Lung Transplantation in Patients With Combined Pulmonary Fibrosis and Emphysema: A Single-Center Experience.

BACKGROUND: Combined pulmonary fibrosis and emphysema (CPFE) is a distinct clinical entity that can progress to end-stage lung disease. Patients with CPFE may develop pulmonary hypertension and face a predicted 1-year mortality of 60%. Lung transplantation is the only curative therapeutic option for CPFE. This report describes our experience after lung transplantation in patients with CPFE. METHODS: This retrospective, single-center study describes short- and long-term outcomes for adult patients who underwent lung transplant for CPFE. RESULTS: The study included 19 patients with explant pathology-proven diagnosis of CPFE. The patients were transplanted between July 2005 and December 2018. Sixteen recipients (84%) had pulmonary hypertension before transplant. Of the 19 patients, 7 (37%) had primary graft dysfunction at 72 hours post-transplant. 1-, 3-, and 5-year freedom from bronchiolitis obliterans syndrome was 100%, 91% (95% CI, 75%-100%), and 82% (95% CI, 62%-100%), respectively. One-, 3-, and 5-year survival was 94% (95% CI, 84%-100%), 82% (95% CI, 65%-100%), and 74% (95% CI, 54%-100%), respectively. CONCLUSION: Our experience demonstrates the safety and feasibility of lung transplant for patients with CPFE. Significant morbidity and mortality without lung transplant coupled with favorable post-transplant outcomes merit prioritization of CPFE in the Lung Allocation Score algorithm for lung transplant candidacy.

Endobronchial valves: 1st Multicenter retrospective study on the 2-step approach.

BACKGROUND: Although the endobronchial valves (EBV) were successfully developed as treatment for severe emphysema, its main complication, pneumothorax, remained an important concern. OBJECTIVE: To assess whether the placement of Zephyr© endobronchial valves throughout 2 procedures instead of 1 minor the frequency of pneumothorax without lowering the benefits of such treatment. METHODS: This retrospective study was conducted in 15 pulmonology department in France. All the patients met the inclusion criteria of the recommendation set by the expert panel on the Endoscopic Lung Volume Reduction (ELVR) updated in 2019. As recommended, all the scan were analyzed with the StratX© (PulmonX Corporation, Redwood city, CA) protocol, and completed by a Chartis© (PulmonX Corporation, Redwood city, CA) in case of questionable fissure. During the first procedure, all but the most proximal sub-segment of the targeted lobe were occluded. One month after, EBV were placed in the bronchus of the last subsegment. All patients were evaluated before and 3 months after the second procedure. RESULTS: Between March 2019 and December 2020, 96 patients received EBV treatment. 12 patients (12.5%) presented a pneumothorax (3 after the 1st step and 9 after the 2nd procedure). Beside pneumothorax, the main adverse event was exacerbation (10.4%) and pneumonia (4.1%). No death were reported. Significant improvement were found for FEV1 (14.6 ± 25.3%), RV (- 0.69 ± 2.1 L), 6MWT (34.8 ± 45.9 m), BODE Score (-1.41 ± 1.41pts), and mMRC scale (-0.85 ± 0.7pts). These results are compared not only to the results previously published using the usual approach but also to our previous publication evaluating the 2-step approach. Some patients presented authentic segmental atelectasis despite infralobar treatment. CONCLUSION: Placing EBV during 2 procedures instead of one led to a significant decrease of post treatment pneumothoraces without increasing the rate of other complications. It does not seem to alter the benefits of such therapy for severe emphysema. These results must be confirmed by launching a multicenter, prospective, randomized, controlled study to compare the frequency of pneumothorax and the efficacy of this new approach with the usual one-time procedure.

Understanding the Impact of Pulmonary Rehabilitation on Airway Resistance in Patients with Severe COPD: A Single-Center Retrospective Study.

Purpose: We investigated the effect of pulmonary rehabilitation (PR) on airway resistance in chronic obstructive pulmonary disease (COPD) patients with severe airway obstruction and hyperinflation. Patients and Methods: This retrospective cohort study was conducted with data from severe COPD cases with those who underwent an 8-week PR program. Main inclusion criteria were having severe airflow obstruction (defined as a forced expiratory volume in one second (FEV1) <50%) and plethysmographic evaluation findings being compatible with hyperinflation supporting the diagnosis of emphysema (presence of hyperinflation defined as functional residual capacity ratio of residual volume to total lung capacity (RV/TLC) >120%). Primary outcomes were airway resistance (Raw) and airway conductance (Gaw) which were measured by body plethysmography, and other measurements were performed, including 6-minute walk test (6-MWT), modified Medical Research Council dyspnea scale (mMRC) and COPD assessment test (CAT). Results: Twenty-six severe and very severe COPD patients (FEV1, 35.0 ± 13.1%; RV/TLC, 163.5 ± 29.4) were included in the analyses, mean age 62.6 ± 5.8 years and 88.5% males. Following rehabilitation, significant improvements in total specific airway resistance percentage (sRawtot%, p = 0.040) and total specific airway conductance percentage (sGawtot%; p = 0.010) were observed. The post-rehabilitation mMRC scores and CAT values were significantly decreased compared to baseline results (p < 0.001 and p < 0.001, respectively). Although there were significant improvements in 6-MWT value (p < 0.001), exercise desaturation (ΔSaO2, p = 0.026), the changes in measured lung capacity and volume values were not significant. Conclusion: We concluded that PR may have a positive effect on airway resistance and airway conductance in COPD patients with severe airflow obstruction.

Association between asthma, chronic bronchitis, emphysema, chronic obstructive pulmonary disease, and lung cancer in the US population.

Lung cancer is one of the primary causes of death with poor life expectancy after diagnosis. History of past respiratory diseases such as asthma, chronic obstructive lung disease (COPD), emphysema, and chronic bronchitis can increase the risk of lung cancer. Very few studies are available to simultaneously assess multiple respiratory diseases and lung cancer. The objective of this study was to investigate correlations between asthma, emphysema, chronic bronchitis, and chronic obstructive lung disease with lung cancer in the US adult population. This was a cross-sectional study using data from a total of 23,523 adult participants from the National Health Examination and Nutrition Survey (NHANES) datasets for seven cycles ranging from 2003-2004 to 2015-2016. To analyze the data, specialized weighted complex survey logit regressions were conducted. Linear logit regression models using only main-effects were constructed first to assess the correlation between the selected demographic and lifestyle variables and asthma, emphysema, chronic bronchitis, and COPD. A second set of linear, main-effects logit regression models were constructed to examine the correlation between lung cancer and asthma, emphysema, chronic bronchitis, COPD when corrected for the selected covariates. The study identified positive correlations between emphysema, chronic bronchitis, COPD, and lung cancer. No correlation between asthma and lung cancer was established. Of the covariates studied, race/ethnicity, marital status, highest educational level, age, family income to poverty ratio, and lifetime smoking were also found to be correlated with the presence of lung cancer. Correlations between the covariates gender, body mass index, alcohol consumption, and country of birth and lung cancer were not found. The study established statistically significant correlations between lung cancer and the lung diseases emphysema, chronic bronchitis, and COPD. The lack of association between asthma and lung cancer may arise from the timeline of diagnosis asthma or type of lung cancer. The study also established significant correlations between lung cancer and several of the covariates included in the analysis. It also established correlations between the covariates and the lung diseases asthma, emphysema, chronic bronchitis, and COPD.

Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry.

BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) is a rare disease that is associated with an increased risk of pulmonary emphysema. The European AATD Research Collaboration (EARCO) international registry was founded with the objective of characterising the individuals with AATD and investigating their natural history. METHODS: The EARCO registry is an international, observational and prospective study of individuals with AATD, defined as AAT serum levels < 11 µM and/or proteinase inhibitor genotypes PI*ZZ, PI*SZ and compound heterozygotes or homozygotes of other rare deficient variants. We describe the characteristics of the individuals included from February 2020 to May 2022. RESULTS: A total of 1044 individuals from 15 countries were analysed. The most frequent genotype was PI*ZZ (60.2%), followed by PI*SZ (29.2%). Among PI*ZZ patients, emphysema was the most frequent lung disease (57.2%) followed by COPD (57.2%) and bronchiectasis (22%). Up to 76.4% had concordant values of FEV1(%) and KCO(%). Those with impairment in FEV1(%) alone had more frequently bronchiectasis and asthma and those with impairment in KCO(%) alone had more frequent emphysema and liver disease. Multivariate analysis showed that advanced age, male sex, exacerbations, increased blood platelets and neutrophils, augmentation and lower AAT serum levels were associated with worse FEV1(%). CONCLUSIONS: EARCO has recruited > 1000 individuals with AATD from 15 countries in its first 2 years. Baseline cross sectional data provide relevant information about the clinical phenotypes of the disease, the patterns of functional impairment and factors associated with poor lung function. Trial registration www. CLINICALTRIALS: gov (ID: NCT04180319).

Prediction of exercise-induced desaturation in COPD patients without resting hypoxemia: a retrospective study.

BACKGROUND: There is no universally accepted criterion for assessing exercise-induced desaturation (EID). The purpose of this study is to compare the two methods regularly used for determining EID in COPD patients, as well as to explore the risk factors and predictors related to EID. METHODS: The 6MWT was performed with continuous SpO2 monitoring on patients with stable COPD. Using two methods (method A: "SpO2rest-SpO2min ≥ 4% and/or SpO2min < 90%", method B: "SpO2rest-SpO2end ≥ 4% and/or SpO2end < 90%") as EID determination criteria to assess the incidence of EID. The differences and consistency of the two methods are compared. Moreover, we collected data through the pulmonary function test, mMRC dyspnea score, COPD assessment test, BODE index and CT-defined emphysema. Univariate and multivariate logistic regression analyses were used to identify factors affecting the EID. For the parameters that predict EID in 6MWT, a receiver operating characteristic (ROC) curve analysis was employed. RESULTS: The analysis included 124 patients. The overall incidence of EID was 62.1% by using method A as the criterion and 51.6% by method B. All of the EID patients found by method B were included in the EID patients identified by method A, as well as 13 new-EID patients. The difference in diagnostic outcomes between the two approaches was not statistically significant (P > 0.05), but they were in excellent agreement (Kappa = 0.807, P = 0.001). Logistic regression analyses found that DLCO SB% pred, DLCO/VA% pred, CAT score, mean density, PD15, emphysema volume and %LAA were significant determinants of the EID. For predicting EID, the ROC analysis produced AUC and cutoffs of 0.689 and 50.45% (DLCO SB% pred), 0.707 and 75.0% (DLCO/VA% pred), 0.727 and 15 points (CAT score), 0.691 and - 955.00HU (PD15), 0.671 and - 856.46HU (mean density), 0.668 and 338.14 ml (emphysema volume) and 0.656 and 7.63% (%LAA), respectively. CONCLUSIONS: Two methods evaluating EID in this research are in a good agreement, method A can find more EID patients by focusing on SpO2min. When conditions are constrained, it is also sufficient to assess EID in COPD patients by method B. In terms of the predictors of EID, DLCO SB% pred, DLCO/VA% pred, CAT score and CT-defined emphysema are all statistically significant test variables to determine EID.

A child presenting with bullous emphysema.

BACKGROUND: Placental transmogrification of the lung (PTL) is a clinical spectrum varying from asymptomatic to severe pulmonary impairment; such as recurrent pneumothorax, bronchopneumonia, respiratory distress syndrome and chronic obstructive airway disease. PTL usually presents as a bullous lesion, and rarely can appear in nodule or cyst formation on chest imaging. PTL with giant bullous emphysema has a male preference, is more commonly unilateral and mostly affects one lobe, but can rarely involve more than one lobe. CASE: Here we report a 13-year-old boy presenting with bullous emphysema and coexisting with a borderline testicular tumor. He had no complaints of cough, sputum, or shortness of breath. He had a past medical history of pneumonia five years ago. In order to elucidate the underlying lung pathology, a wedge lung biopsy was performed and the patient was diagnosed with PTL. Scrotum ultrasonography was performed because of hydrocele in both testes, and bilateral epididymal cysts with papillary solid projections were reported. Pathological examination of the epididymal tumor revealed a `Mullerian type borderline epithelial neoplasm` which is an analogue of the ovarian serous borderline tumor. CONCLUSIONS: In conclusion, we reported the youngest PTL case in the literature, a rare disease with unknown pathophysiology, presenting as bullous emphysema and coincidental Mullerian type borderline epithelial neoplasm. It is important to diagnose placental transmogrification of the lung in a child with bullous emphysema because compared to other cystic lung diseases it is a benign disease and if no additional malignity exists, lobectomy or pneumonectomy is the cure for the disease.