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Malignant central airway obstruction (MCAO) is a debilitating and life-limiting complication that occurs in an unfortunately large number of individuals with advanced intrathoracic cancer. Although the management of MCAO is multimodal and interdisciplinary, the task of providing patients with prompt palliation falls increasingly on the shoulders of interventional pulmonologists. While a variety of tools and techniques are available for the management of malignant obstructive lesions, advancements and evolution in this therapeutic venue have been somewhat sluggish and limited when compared with other branches of interventional pulmonary medicine (e.g., the early diagnosis of peripheral lung nodules). Indeed, one pragmatic, albeit somewhat uncharitable, reading of this article's title might suggest a wry smile and shug of the shoulders as to imply that relatively little has changed in recent years. That said, the spectrum of interventions for MCAO continues to expand, even if at a less impressive clip. Herein, we present on MCAO and its endoscopic and nonendoscopic management-that which is old, that which is new, and that which is still on the horizon.

Endoscopic therapies are firmly established in the management algorithm of benign subglottic and tracheal stenosis (SGTS). The optimal dilation strategy, however, has yet to be elucidated. The objective of this study was to compare the efficacy and safety of balloon versus rigid bronchoplasty in the treatment of benign SGTS.De novo cases of benign SGTS at our institution over a 9-year period were retrospectively identified. Patients were divided into 2 groups based on the initial dilation strategy of balloon or rigid bronchoplasty. Demographics, clinical findings, concurrent interventions, lesion characteristics, and complications were analyzed. Two reviewers independently assigned an index and follow-up endoscopic stenosis grade for each case. The mean stenosis grade at follow-up in both groups was then calculated and compared.Sixty-three patients with benign SGTS were included. Most stenoses in the rigid (80%) and balloon (63%) bronchoplasty groups were complex ( P =0.174). In addition, 94% (59/63) of index stenoses were classified as Cotton Myer Grade 3. At follow-up, no significant difference was found in the mean stenosis grade between dilation strategies (1.97 vs. 2.2, P =0.287). Furthermore, no procedural-related complications were observed in either group.Balloon and rigid bronchoplasty are safe and effective endoscopic tools in the early management of benign SGTS. A multimodality approach centered around mucosal sparing techniques remains vitally important to the overall and likely long-term success of treating this challenging disease entity.

Electromagnetic navigation bronchoscopy (ENB) is a minimally invasive, image-guided approach to access lung lesions for biopsy or localization for treatment. However, no studies have reported prospective 24-month follow-up from a large, multinational, generalizable cohort. This study evaluated ENB safety, diagnostic yield, and usage patterns in an unrestricted, real-world observational design.The NAVIGATE single-arm, pragmatic cohort study (NCT02410837) enrolled subjects at 37 academic and community sites in seven countries with prospective 24-month follow-up. Subjects underwent ENB using the superDimension navigation system versions 6.3 to 7.1. The prespecified primary end point was procedure-related pneumothorax requiring intervention or hospitalization.A total of 1388 subjects were enrolled for lung lesion biopsy (1329; 95.7%), fiducial marker placement (272; 19.6%), dye marking (23; 1.7%), or lymph node biopsy (36; 2.6%). Concurrent endobronchial ultrasound-guided staging occurred in 456 subjects. General anesthesia (78.2% overall, 56.6% Europe, 81.4% United States), radial endobronchial ultrasound (50.6%, 4.0%, 57.4%), fluoroscopy (85.0%, 41.7%, 91.0%), and rapid on-site evaluation use (61.7%, 17.3%, 68.5%) differed between regions. Pneumothorax and bronchopulmonary hemorrhage occurred in 4.7% and 2.7% of subjects, respectively (3.2% [primary end point] and 1.7% requiring intervention or hospitalization). Respiratory failure occurred in 0.6%. The diagnostic yield was 67.8% (range: 61.9%-70.7%; 55.2% Europe, 69.8% United States). Sensitivity for malignancy was 62.6%. Lung cancer clinical stage was I to II in 64.7% (55.3% Europe, 65.8% United States).Despite a heterogeneous cohort and regional differences in procedural techniques, ENB demonstrates low complications and a 67.8% diagnostic yield while allowing biopsy, staging, fiducial placement, and dye marking in a single procedure.

Building an efficient facility for advanced bronchoscopic procedures involves many considerations. This review places particular emphasis on anesthesiology services, based on experience at a tertiary/quaternary care referral academic medical center. Topics include equipment requirements, applicable clinical standards and multidisciplinary collaboration. Patient flow arrangements for both outpatients and inpatients, from pre-operative care to discharge/disposition are highlighted. The importance of effective business planning, personnel training, leadership, communication, teambuilding, quality of care and patient safety are also discussed.

A central goal of an airway stent is to restore patency by preventing restenosis, holding the tracheobronchial wall, or occluding fistulas. Complications with stents, however, are frequent and can have grave repercussions. Stents are therefore viewed as a last resort in cases where other forms of treatment are ineffective. Furthermore, it is common for people with complex airways to have airway stents that do not fit them well, which can result in several complications. Three-dimensional printing technology was developed at the turn of the 20th century. It has been employed in a variety of applications and has transformed healthcare. This technology has mainly been employed in respiratory medicine to develop three-dimensional models of the airways and to make airway splints and prostheses to treat central airway diseases. In the past ten years, it has transformed and advanced personalized medicine, enabling the creation of patient-specific stents for people with complex airway diseases. Three-dimensional printing might be used to create a patient-specific stent that would lessen risks, enhance the quality of life, and eliminate the need for additional procedures. This chapter discusses the most recent developments in three-dimensional printing technology, how they are being used to create airway prostheses to treat complex airway illnesses and the current body of research that supports their use.

Background Two models, the Help with the Assessment of Adenopathy in Lung cancer (HAL) and Help with Oncologic Mediastinal Evaluation for Radiation (HOMER), were recently developed to estimate the probability of nodal disease in patients with non-small cell lung cancer (NSCLC) as determined by endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA). The objective of this study was to prospectively externally validate both models at multiple centers. Research Question Are the HAL and HOMER models valid across multiple centers? Study Design and Methods This multicenter prospective observational cohort study enrolled consecutive patients with PET-CT clinical-radiographic stages T1-3, N0-3, M0 NSCLC undergoing EBUS-TBNA staging. HOMER was used to predict the probability of N0 vs N1 vs N2 or N3 (N2|3) disease, and HAL was used to predict the probability of N2|3 (vs N0 or N1) disease. Model discrimination was assessed using the area under the receiver operating characteristics curve (ROC-AUC), and calibration was assessed using the Brier score, calibration plots, and the Hosmer-Lemeshow test. Results Thirteen centers enrolled 1,799 patients. HAL and HOMER demonstrated good discrimination: HAL ROC-AUC = 0.873 (95%CI, 0.856-0.891) and HOMER ROC-AUC = 0.837 (95%CI, 0.814-0.859) for predicting N1 disease or higher (N1|2|3) and 0.876 (95%CI, 0.855-0.897) for predicting N2|3 disease. Brier scores were 0.117 and 0.349, respectively. Calibration plots demonstrated good calibration for both models. For HAL, the difference between forecast and observed probability of N2|3 disease was +0.012; for HOMER, the difference for N1|2|3 was −0.018 and for N2|3 was +0.002. The Hosmer-Lemeshow test was significant for both models (P = .034 and .002), indicating a small but statistically significant calibration error. Interpretation HAL and HOMER demonstrated good discrimination and calibration in multiple centers. Although calibration error was present, the magnitude of the error is small, such that the models are informative.

Advanced interventional pulmonary procedures of the airways, pleural space, and mediastinum continue to evolve and be refined. Health care, finance, and clinical professionals are challenged by both the indications and related coding complexities. As the scope of interventional pulmonary procedures expands with advanced technique and medical innovation, program planning and ongoing collaboration among clinicians, finance executives, and reimbursement experts are key elements for success. We describe advanced bronchoscopic procedures, appropriate Current Procedural Terminology coding, valuations, and necessary modifiers to fill the knowledge gap between basic and advanced procedural coding. Our approach is to balance the description of procedures with the associated coding in a way that is of use to the proceduralist, the coding specialist, and other nonclinical professionals.

Background The diagnosis of peripheral pulmonary lesions (PPL) continues to present clinical challenges. Despite extensive experience with guided bronchoscopy, the diagnostic yield has not improved significantly. Robotic-assisted bronchoscopic platforms have been developed potentially to improve the diagnostic yield for PPL. Presently, limited data exist that evaluate the performance of robotic systems in live human subjects. Research Question What is the safety and feasibility of robotic-assisted bronchoscopy in patients with PPLs? Study Design and Methods This was a prospective, multicenter pilot and feasibility study that used a robotic bronchoscopic system with a mother-daughter configuration in patients with PPL 1 to 5 cm in size. The primary end points were successful lesion localization with the use of radial probe endobronchial ultrasound (R-EBUS) imaging and incidence of procedure related adverse events. Robotic bronchoscopy was performed in patients with the use of direct visualization, electromagnetic navigation, and fluoroscopy. After the use of R-EBUS imaging, transbronchial needle aspiration was performed. Rapid on-site evaluation (ROSE) was used on all cases. Transbronchial needle aspiration alone was sufficient when ROSE was diagnostic; when ROSE was not diagnostic, transbronchial biopsy was performed with the use of the robotic platform, followed by conventional guided bronchoscopic approaches at the discretion of the investigator. Results Fifty-five patients were enrolled at five centers. One patient withdrew consent, which left 54 patients for data analysis. Median lesion size was 23 mm (interquartile range, 15 to 29 mm). R-EBUS images were available in 53 of 54 cases. Lesion localization was successful in 51 of 53 patients (96.2%). Pneumothorax was reported in two of 54 of the cases (3.7%); tube thoracostomy was required in one of the cases (1.9 %). No additional adverse events occurred. Interpretation This is the first, prospective, multicenter study of robotic bronchoscopy in patients with PPLs. Successful lesion localization was achieved in 96.2% of cases, with an adverse event rate comparable with conventional bronchoscopic procedures. Additional large prospective studies are warranted to evaluate procedure characteristics, such as diagnostic yield. Clinical Trial Registration ClinicalTrials.gov; No.: NCT03727425; URL: www.clinicaltrials.gov .

The peripheral pulmonary nodule offers unique challenges to the clinician, especially in regards to diagnostic approach. Quite often the etiology of the nodule is spurious, though the specter of malignancy drives accurate classification of the nodule. Diagnostic approaches range in degrees of invasiveness, accuracy, and morbidity. Bronchoscopic access to these nodules had been plagued by low reported yields, especially in fluoroscopically invisible nodules. Navigational bronchoscopy, however, allowed more accurate access to peripheral nodules while maintaining a low morbidity, and thus reshaped the historic diagnostic algorithms. Though navigational bronchoscopy was initially associated with electromagnetic navigation, newer approaches to navigation and new technologies provide enthusiasm that yield can improve. In this article we will provide a historical approach to navigational bronchoscopy, from its origins to its current state, and we will discuss developing technology and its potential role in the evolving paradigm of the peripheral nodule biopsy.

Traditional therapies for emphysema such as bronchodilators and anti-inflammatory drugs have limited value due to permanent structural changes in the emphysematous lung that result in hyperinflation. Surgical lung volume reduction partially corrects hyperinflation by removing emphysematous lung and is an option in selected patients, but it carries a risk of morbidity and death. Valve therapy is a less-invasive option that involves bronchoscopic implantation of 1-way valves in emphysematous lung segments to allow air flow and mucus clearance in the direction of central airways. The authors review the rationale, evidence, and applications of valve therapy.

Lung nodules are being identified with increasing frequency. With this growing burden of nodules comes a growing need for diagnostic technologies extending beyond the current reach of conventional bronchoscopy. One such method for diagnosing peripheral lung lesions is electromagnetic navigational bronchoscopy (ENB), which comprises a set of tools designed to aid the bronchoscopist in identifying, accessing, and sampling peripheral lung lesions under virtual guidance.Herein we present an in-depth review of ENB, including commercially available electromagnetic navigation platforms, factors influencing diagnostic yield, adjunctive imaging and biopsy tools, potential risks, cost, technical shortcomings, and competing technologies. A review of the scientific literature was conducted primarily through PubMed, ScienceDirect, and Google Scholar, and pertinent publications and abstracts from the inception of electromagnetic navigation through early 2020 were considered. We also share our perspective on the future of ENB from both a diagnostic and a therapeutic standpoint.ENB is currently a leading tool in the diagnostic evaluation of peripheral lung lesions. The future of ENB rests not only on its potential to expand into the therapeutic realm but also on its ability to keep pace with competing diagnostic and therapeutic technologies.

Bronchoscopy is essential to the practice of pulmonary medicine. It is an important diagnostic and therapeutic tool for many disease processes. Bronchoscopy can be performed in a variety of clinical settings, from the bedside to an operating room. Although bronchoscopy has been practiced for more than a century, consensus recommendations from stakeholders have yet to be developed for the planning, implementation, and construction of a bronchoscopy suite. A wide range of procedures can be performed via bronchoscopy; therefore, the required tools and the procedure area must be aligned with the needs of the facility. Designing a bronchoscopy suite is by no means a "one size fits all" process. We present an overview of critical features to be considered in the planning for an ideal bronchoscopy suite. We use the term "ideal" because it represents a subjective conception of what is perfect and does not convey a rigid, universal blueprint.

Rationale: When stereotactic ablative radiotherapy is an option for patients with non–small cell lung cancer (NSCLC), distinguishing between N0, N1, and N2 or N3 (N2|3) disease is important.Objecti...

Background Foregut cysts account for >50% of cystic lesions in the mediastinum, of which bronchogenic cysts are most common. Surgical resection is the most definitive approach for its diagnosis and treatment. A recent systematic review, however, suggests that endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has a role in the management of bronchogenic cyst. We report our experience with EBUS-TBNA in the diagnosis and management of bronchogenic cysts. Methods Medical records of patients with evidence of mediastinal cysts who underwent EBUS-TBNA between 2008 and 2016 were reviewed.The primary aims of this study were to assess EBUS-TBNA diagnostic yield of peri-bronchial cysts and their specific type/origin and to determine its short-term and long-term drainage efficacy. Results A total of 26 patients met the inclusion criteria. The cytopathology diagnosis was compatible with bronchogenic cyst in 4 cases, pleural-pericardial cyst in 3 cases, and 19 were indeterminate cysts. Successful long-term treatment occurred in 5.5% of the subjects. One patient developed inflammatory pericarditis after EBUS-TBNA. Conclusion Diagnostic and therapeutic yield of EBUS-TBNA for mediastinal cysts is limited and surgical resection remains the treatment of choice.

BACKGROUND The Bonastent is one of the newest self-expanding metallic tracheobronchial prostheses being used in the central airways. Experience with this stent is limited; there is a gap in literature. We report herein a case series of Bonastent implantation across a variety of tracheobronchial pathologies. METHODS All adult patients who had a Bonastent implanted within the tracheobronchial tree between November 2017 through May 2018 were included in this case series. Data were derived from a combination of sources: electronic medical records and a Research Electronic Data Capture-based bronchoscopy registry. Demographic, procedural, and short-term outcome data were collected for each patient. RESULTS Thirteen Bonastents were implanted in 11 patients during the observational period. Seven patients were male (64%); the mean age was 62.2 years. The most common indication was nonmalignant airway obstruction in a transplanted lung (n=6, 56%). Over half of all stents (n=8) were implanted in the bronchus intermedius. Three patients (27%) had clinically significant obstruction from their Bonastent. CONCLUSION The Bonastent is the newest self-expanding metallic endotracheobronchial stent on the market. Like any other stent, it has its advantages and drawbacks. Interestingly, we noted 3 instances of tissue ingrowth through the covered portion of the stent. Larger studies with a longer follow-up periods are needed to assess long-term efficacy and safety of this relatively novel stent.

Introduction Electromagnetic navigation bronchoscopy (ENB) is a minimally invasive technology that guides endoscopic tools to pulmonary lesions. ENB has been evaluated primarily in small, single-center studies; thus, the diagnostic yield in a generalizable setting is unknown. Methods NAVIGATE is a prospective, multicenter, cohort study that evaluated ENB using the superDimension navigation system (Medtronic, Minneapolis, Minnesota). In this United States cohort analysis, 1215 consecutive subjects were enrolled at 29 academic and community sites from April 2015 to August 2016. Results The median lesion size was 20.0 mm. Fluoroscopy was used in 91% of cases (lesions visible in 60%) and radial endobronchial ultrasound in 57%. The median ENB planning time was 5 minutes; the ENB-specific procedure time was 25 minutes. Among 1157 subjects undergoing ENB-guided biopsy, 94% (1092 of 1157) had navigation completed and tissue obtained. Follow-up was completed in 99% of subjects at 1 month and 80% at 12 months. The 12-month diagnostic yield was 73%. Pathology results of the ENB-aided tissue samples showed malignancy in 44% (484 of 1092). Sensitivity, specificity, positive predictive value, and negative predictive value for malignancy were 69%, 100%, 100%, and 56%, respectively. ENB-related Common Terminology Criteria for Adverse Events grade 2 or higher pneumothoraces (requiring admission or chest tube placement) occurred in 2.9%. The ENB-related Common Terminology Criteria for Adverse Events grade 2 or higher bronchopulmonary hemorrhage and grade 4 or higher respiratory failure rates were 1.5% and 0.7%, respectively. Conclusions NAVIGATE shows that an ENB-aided diagnosis can be obtained in approximately three-quarters of evaluable patients across a generalizable cohort based on prospective 12-month follow-up in a pragmatic setting with a low procedural complication rate.

Tracheobronchial injury is a rare but a potentially high-impact event with significant morbidity and mortality. Common etiologies include blunt or penetrating trauma and iatrogenic injury that might occur during surgery, endotracheal intubation, or bronchoscopy. Early recognition of clinical signs and symptoms can help risk-stratify patients and guide management. In recent years, there has been a paradigm shift in the management of tracheal injury towards minimally invasive modalities, such as endobronchial stent placement. Although there are still some definitive indications for surgery, selected patients who meet traditional surgical criteria as well as those patients who were deemed to be poor surgical candidates can now be managed successfully using minimally invasive techniques. This paradigm shift from surgical to nonsurgical management is promising and should be considered prior to making final management decisions.

Building an efficient facility for advanced bronchoscopic procedures involves many considerations. This review places particular emphasis on anesthesiology services, based on experience at a tertiary/quaternary care referral academic medical center. Topics include equipment requirements, applicable clinical standards, and multidisciplinary collaboration. Patient flow arrangements for both outpatients and inpatients, from preoperative care to discharge/disposition, are highlighted. The importance of effective business planning, personnel training, leadership, communication, team building, quality of care, and patient safety are also discussed.

Bronchoscopy is an aerosol-generating procedure that creates unique challenges for healthcare providers to reduce the potential spread of the COVID-19 respiratory pathogen. As part of the initial response, Cleveland Clinic postponed elective surgeries including bronchoscopy. We established a 5-tier system for prioritizing the urgency of bronchoscopy procedures. When elective bronchoscopies were resumed, we established protocols to reduce aerosolization and potential virus transmission risks such as using an airborne infection-isolation room and changing to total intravenous anesthesia. Also, we established guidelines for periprocedural care and use of personal protective equipment including requirements for wearing N95 masks for all bronchoscopy procedures.

Background The diagnostic yield of electromagnetic navigation bronchoscopy (ENB) is impacted by biopsy tool strategy and rapid on-site evaluation (ROSE) use. This analysis evaluates usage patterns, accuracy, and safety of tool strategy and ROSE in a multicenter study. Methods NAVIGATE (NCT02410837) evaluates ENB using the superDimension navigation system (versions 6.3 to 7.1). The 1-year analysis included 1215 prospectively enrolled subjects at 29 United States sites. Included herein are 416 subjects who underwent ENB-aided biopsy of a single lung lesion positive for malignancy at 1 year. Use of a restricted number of tools (only biopsy forceps, standard cytology brush, and/or bronchoalveolar lavage) was compared with an extensive multimodal strategy (biopsy forceps, cytology brush, aspirating needle, triple needle cytology brush, needle-tipped cytology brush, core biopsy system, and bronchoalveolar lavage). Results Of malignant cases, 86.8% (361/416) of true positive diagnoses were obtained using extensive multimodal strategies. ROSE was used in 300/416 cases. The finding of malignancy by ROSE reduced the total number of tools used. A malignant ROSE call was obtained in 71% (212/300), most (88.7%; 188/212) by the first tool used (49.5% with aspirating needle, 20.2% with cytology brush, 17.0% with forceps). True positive rates were highest for the biopsy forceps (86.9%) and aspirating needle (86.6%). Use of extensive tool strategies did not increase the rates of pneumothorax (5.5% restricted, 2.8% extensive) or bronchopulmonary hemorrhage (3.6% restricted, 1.1% extensive). Conclusion These results suggest that extensive biopsy tool strategies, including the aspirating needle, may provide higher true positive rates for detecting lung cancer without increasing complications.

Advanced interventional pulmonary procedures of the airways, pleural space, and mediastinum continue to evolve and be refined. Health care, finance, and clinical professionals are challenged by both the indications and related coding complexities. As the scope of interventional pulmonary procedures expands with advanced technique and medical innovation, program planning and ongoing collaboration among clinicians, finance executives, and reimbursement experts are key elements for success. We describe advanced bronchoscopic procedures, appropriate Current Procedural Terminology coding, valuations, and necessary modifiers to fill the knowledge gap between basic and advanced procedural coding. Our approach is to balance the description of procedures with the associated coding in a way that is of use to the proceduralist, the coding specialist, and other nonclinical professionals.

Die Behandlung komplexer gutartiger Atemwegserkrankungen stellt eine große Herausforderung für die interventionelle Pneumologie dar. Stents in den Atemwegen können aus verschiedenen Gründen Komplikationen hervorrufen, unter anderem bei mangelhafter Passung des Stents. Wir berichten hier über das 1-Jahres-Outcome von 2 Patienten mit Atemwegserkrankung im Rahmen einer Granulomatose mit Polyangiitis (Morbus Wegener) mit Beteiligung des linken Hauptbronchus und der sekundären Carina. Bei beiden Patienten hatten systemische Therapien und bronchoskopische Standardverfahren versagt und handelsübliche Atemwegs-Stents zu Komplikationen geführt. Wir beschreiben unsere klinische Erfahrung mit einem erstmals beim Menschen angewandten Ansatz zur Überwindung dieser Probleme: Mittels Computertomographie- (CT-)Bildgebung und 3D-Drucktechnologie stellten wir einen patientenspezifischen Atemwegs-Stent aus Silikon her und implantierten diesen bei den Patienten mit Atemwegserkrankung. Den Bauplan oder das Rezept für den patientenspezifischen Stent erstellten wir anhand von Thorax-CT-Aufnahmen der Patienten in einem proprietären Softwarepaket, das ursprünglich für die orthopädische OP-Planung entwickelt worden war. Die Silikon-Stents wurden angefertigt und implantiert. Die Nachbeobachtung über >1 Jahr nach der Implantation des patientenspezifischen Stents wurde mit den 6 Monaten vor der Implantation verglichen; Kriterien waren die Anzahl klinisch erforderlicher Stent-Wechsel, die Behandlungsdauer und die allgemeine klinische Verbesserung unter standardmäßiger Versorung nach einem Atemwegs-Stenting. Der Vergleich ergab längere Stent-Liegezeit, kürzere Behandlungsdauer sowie Besserung der Symptome laut Patientenauskunft und dadurch geringeren Bedarf an Stent-Wechseln und -Modifikationen. Die Herstellung patientenspezifischer Silikon-Stents mittels 3D-Druck ist mithin durchführbar, und der erste klinische Nachweis der Praxistauglichkeit (Proof of Concept) belegt eine dauerhafte Verbesserung über den einjährigen Nachbeobachtungszeitraum.

Managing complex benign airway disease is a major challenge in interventional pulmonology. Airway stent placement can result in complications due to a variety of factors, including poor fit of the stent in the airway. We report the 1-year outcome of 2 patients with airway disease caused by granulomatosis with polyangiitis (Wegener’s) affecting the left main bronchus and secondary carina. These patients had not responded to systemic therapy or standard bronchoscopic techniques and had complications with commercially available airway stents. We describe a first-in-human, clinical experience to address these issues. Using computed tomography (CT) imaging and 3D printing technology, we generated and implanted patient-specific silicone airway stents to address airway disease. 3D patient-specific stent prescription was created using a CT scan of the patient’s chest and a proprietary software package originally developed for orthopedic surgical planning. Silicone stents were manufactured and implanted. Observation for > 1 year after implantation compared to the 6 months prior to patient-specific stent implant were compared for the number, clinically required stent changes, procedure time, and general clinical improvement per usual standard of care after airway stenting. Patients showed improved durability, a shorter procedure time, and improvement of patient-reported symptoms leading to a reduced need for stent changes and modifications. The use of 3D printing technology to make patient-specific silicone stents is feasible with early clinical proof of concept noting a durable improvement over 1 year of follow-up.

Fungal infections of the lung are commonly seen by practicing pathologists. Typically caused by inhalation of fungal organisms (spores or hyphae), these infections can be broadly classified into two categories: endemic fungi, which typically infect immunocompetent hosts (but may also infect immunocompromised patients), and opportunistic fungi, which only infect immunocompromised patients. This chapter reviews six of the most common lung fungal infections seen in North America.

Systemic diseases commonly affect the lung, producing secondary pathology that can be difficult to recognize from primary lung diseases. These include pulmonary involvement by various autoimmune diseases such as relapsing polychondritis, rheumatoid arthritis involvement in the form of rheumatoid nodules, IgG4-related sclerosing disease, diseases that arise within compromised immune systems such as granulomatous lymphocytic interstitial lung disease (GLILD), and deposition diseases such as amyloid and light chain deposition disease. This chapter describes the clinical and pathology features of these six entities so that practicing pathologists recognize them.

The category of small airway diseases applies to three major groups of lung diseases. These include respiratory bronchiolitis, follicular bronchiolitis, and constrictive bronchiolitis. Though many inflammatory diseases of the lung have small airway pathology as a secondary component and descriptions of those will be found in other chapters (interstitial lung diseases, pneumoconiosis, and infections), in these three diseases, the small airway pathology represents the primary pathology seen in the lung. These pathologic diseases are characterized by the common clinical features of shortness of breath, cough, decreased expiratory volumes, and/or hypoxia. These are found in multiple clinical settings including smoking/environmental exposures, immune deficiencies, and transplantation, the latter of which includes both primary rejection of the lung allograft and graft-versus-host disease in the lung secondary to bone marrow transplantation. Though cytopathology evaluation in these diagnoses is limited, both imaging and small biopsies specimens can be helpful, if evaluated within the appropriate clinical context.

Salivary gland-like tumors are rare primary malignancies in the lung. They arise out of the seromucinous glands of the cartilaginous airways in the lung and are morphologically similar to their salivary gland counterparts. Surgical resection offers the best chance for a cure, but the central airway locations make postsurgical complications including airway stenosis and postsurgical dehiscences common. This chapter reviews the two most common tumors in this category, mucoepidermoid carcinoma and adenoid cystic carcinoma.

The interstitial pneumonias consist of pathologic patterns of injury that involve the alveolar septa and associated connective tissue of the lung. These pathologies are commonly found within characteristic clinical settings and have predictable prognoses and some have specific therapies. In general, these pathologic patterns of injury have nonspecific fibroinflammatory features when seen in small biopsies, but have characteristic features when viewed in the context of larger (thoracoscopic) biopsies and are most accurately diagnosed in the setting of a multidisciplinary approach employing clinical, radiologic, and pathologic input.

The mesenchymal tumors of the lung consist of a variety of neoplasms that originate from cells of mesenchymal origin, including endothelium, muscle, fibroblasts, cartilage/bone. The tumors with these pathologic features are most commonly seen in the lung as metastatic lesions from sarcomas elsewhere in the body. However, there are examples of primary mesenchymal tumors in the lung that are important to recognize. The most important of these are listed below. Of note, lymphangioleiomyomatosis was previously considered an interstitial lung disease, but most recent studies have shown it to be a neoplasm arising from the perivascular epithelioid cells (PECs) of the lung and is appropriately included in this chapter.

The term chronic obstructive pulmonary disease (COPD) applies to three major groups of lung diseases. These include emphysema, asthma/chronic bronchitis, and bronchiectasis. These pathologic diseases are characterized by common clinical features of shortness of breath, cough, decreased expiratory volumes, and hypoxia. There are two major mechanisms that cause the obstructive clinical features of these diseases and are reflected in the pathologic features. Emphysema, a loss of alveolar walls, is caused by the destruction of elastic fibers within the alveolar walls of the pulmonary lobules. This results in a loss of elastic recoil and premature closer of the small airways, limiting the expiratory phase of breathing. Asthma/chronic bronchitis and bronchiectasis obstruct airway flow by narrowing the opening of the lumen. This occurs by increased luminal secretions (mucous, sloughed inflammatory cells), thickening of the airway walls (inflammation, increased smooth muscle, and fibrosis), and increased submucosal glands present in the wall of the airways. Though these diseases are mostly diagnosed clinically, it is worthwhile to be familiar with the underlying pathologic features that cause these symptoms.

Pulmonary hypertensive vascular diseases are diagnosed most frequently clinically, with the help of imaging and invasive cardiovascular testing. Nonetheless, though biopsies for these diseases are rare, these lesions can be encountered in lung biopsies performed to evaluate the patient for other lung diseases, both neoplastic and nonneoplastic. Therefore, the practicing pathologist should become familiar with the characteristic features of these diseases so that they can recognize them. This chapter reviews the vascular changes seen in both low-grade and high-grade pulmonary hypertensive arteriopathy, including the characteristic plexiform and vasodilatation lesions seen in the latter. Also, the pathologic lesions of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are reviewed as these may be subtle and difficult recognize. Not surprisingly, both bronchoscopic images and cytopathology play minimal roles in the diagnoses of these diseases, but thoracic imaging studies are central to making these clinical-pathologic diagnoses.

Carcinoid tumors represent

Lung cancer now represents the leading overall cause of cancer in the world. These neoplasms are predominantly carcinomas, and are commonly caused by cigarette smoking. The three most common histologic types of non-small cell carcinomas – adenocarcinoma, squamous cell carcinoma, and large cell carcinoma – can be distinguishable by morphologic features defined by international classifications, most notably the World Health Organization Classification of the Tumours of the Lung (2015). Though morphology is the mainstay for tissue diagnosis, histochemical and immunohistochemical testing are commonly employed to make these diagnoses, especially in poorly differentiated tumors. Most importantly, molecular testing to define targetable mutations for therapeutic use is employed in these carcinomas and has resulted in improved clinical outcomes.