The authors describe the case of a 54 year-old female with adenoid cystic carcinoma of the trachea at the cricoid level upon whom extensive surgical resection was performed, including the larynx, part of the trachea and esophagus. Reconstruction of the digestive tract was done by means of a gastric tube and the airway with a mediastinal tracheostomy. Five years after the original procedure there was a local cutaneous recurrence which was removed. Seven years later the disease recurred in the mediastinum, which responded to irradiation. The patient is alive and well eight years and five months after the operation.

Keywords: Trachea. Neoplasm. Carcinoma, adenoid cystic. Tracheostomy.

In cases of recent asthma in which clinical control with the usual treatment (corticosteroids and bronchodilator) is unsatisfactory, it is important to consider other diagnoses, such as congestive heart failure, gastroesophageal reflux or other forms of airway obstruction. We report the case of a female patient with mucoepidermoid carcinoma of the trachea mimicking asthma. The patient presented cough and wheezing, as well as abnormal spirometry results with an obstructive pattern that was responsive to bronchodilators. One year later, the patient presented clinical and spirometric worsening. The chest X-ray revealed no abnormalities. A CT scan showed a vegetative lesion obstructing the tracheal lumen and located 1 cm from the carina. Fiberoptic bronchoscopy showed a finding similar to a bronchial carcinoid tumor. The anatomopathological diagnosis made after surgical resection was low-grade mucoepidermoid carcinoma, without lymph node involvement. Although the flow-volume curve was not suggestive of upper airway obstruction, the spirometry performed after the surgery showed a significant reduction in the degree of obstruction and greater reversibility after bronchodilator use. There was no evidence of recurrence of the disease or of the symptoms after a two-year follow-up period.

Keywords: Bronchial hyperreactivity; Carcinoma, mucoepidermoid; Trachea; Asthma; Lung diseases, obstructive.

Objective: To evaluate the effects of exposure to emissions from sugar cane burning on inflammatory mechanisms in tissues of the trachea and lung parenchyma in Wistar rats after different periods of exposure. Methods: This was an experimental open randomized study. The animals were divided into four groups: a control group (CG) underwent standard laboratory conditions, and three experimental groups were exposed to emissions from sugar cane burning over different periods of time, in days-1 (EG1), 7 (EG7), and 21 (EG21). After euthanasia with 200 mg/kg of ketamine/xylazine, fragments of trachea and lung were collected and fixed in 10% formalin. Histological analyses were performed with H&E and picrosirius red staining. Results: No inflammatory infiltrates were found in the tissues of CG rats. The histological examination of tissues of the trachea and lung parenchyma revealed that the inflammatory process was significantly more intense in EG7 than in the CG (p < 0.05 and p < 0.01, respectively). In comparison with the CG and EG1, angiogenesis in the lung parenchyma and collagen deposition in tracheal tissues were significantly greater only in EG21 (p < 0.001 and p < 0.01, respectively). Conclusions: In this sample, emissions from sugar cane burning induced acute focal and diffuse inflammation in the lamina propria of tracheal tissues, with no loss of ciliated epithelial tissue. In the lung parenchyma of the animals in the experimental groups, there was interstitial and alveolar edema, together with polymorphonuclear cell infiltrates.

Keywords: Saccharum; Smoke; Inflammation; Respiratory system.

Objective: To describe the mortality trends for lung cancer, cancer of the trachea, and bronchial cancer in relation to gender and age brackets in Brazil. Methods: Data related to mortality between 1980 and 2003 were collected from the Brazilian Mortality Database. A trend analysis of mortality was carried out, nationwide and in selected states, using the LOWESS technique for rate smoothing and model adjustments. Results: In Brazil, the standardized mortality rate for lung cancer, cancer of the trachea, and bronchial cancer increased from 7.21/100,000 inhabitants in 1980 to 9.36/100,000 inhabitants in 2003. Specific mortality rates decreased in males in the 30-49 and 50-59 age brackets. In the 60-69 age bracket, the rates for males increased from 1980 to 1995 and declined thereafter. There was a trend toward higher mortality rates in males over 70, as well as in females over 30, throughout the period evaluated. Conclusions: The decrease in the mortality rates in younger males might have resulted from recent national interventions aimed at reducing the prevalence of smoking and reducing exposure in younger cohorts. High mortality rates in older populations remained constant due to prior tobacco use. Increased mortality rates in females are a worldwide trend and are attributable to the recent increase in smoking prevalence in females.

Keywords: Lung neoplasms/epidemiology; Mortality/trends; Brazil.

The aim of this pictorial essay was to present the main computed tomography findings seen in diffuse diseases of the trachea. The diseases studied included amyloidosis, tracheobronchopathia osteochondroplastica, tracheobronchomegaly, laryngotracheobronchial papillomatosis, lymphoma, neurofibromatosis, relapsing polychondritis, Wegener's granulomatosis, tuberculosis, paracoccidioidomycosis, and tracheobronchomalacia. The most common computed tomography finding was thickening of the walls of the trachea, with or without nodules, parietal calcifications, or involvement of the posterior wall. Although computed tomography allows the detection and characterization of diseases of the central airways, and the correlation with clinical data reduces the diagnostic possibilities, bronchoscopy with biopsy remains the most useful procedure for the diagnosis of diffuse lesions of the trachea.

Keywords: Thoracic diseases; Tracheal diseases; Tomography, X-ray computed; Trachea.

Granular cell tumors are uncommon neoplasm arising from Schwann cell. They are uncommon in the respiratory tract and there are only 32 cases described with primary tracheal involvement. A case is described of a 28 year-old woman with granular cell tumor located primarily in the trachea. The tumor was treated surgically with good result.

Keywords: granular cell tumor; trachea

Objectives: Thoracoscopic management of mediastinal tumors is still subject to analysis. Seventy-three patients were submitted to thoracoscopy for the treatment of mediastinal masses and were analyzed retrospectively, in order to evaluate the effectiveness and complications of the procedure. Methods: Between 1983 and 1999, 21 conventional thoracoscopies and 52 video-assisted thoracic surgeries were performed (33 for diagnostic purposes and 40 for therapy). Patient ages ranged from two to 81 years (mean 43.8) with a slight predominance of females over males (41 versus 32). All underwent general anesthesia using simple (22) or double lumen (51) intubation. Results: The histological type of tumor was identified in all patients. For therapeutic purposes, conversion to thoracotomy was necessary in nine patients. The reasons were tumor size and invasion of nearby structures, difficulty to continue dissection, to perform an upper lobectomy, and to suture the iatrogenic diaphragm lesion. Four patients died during the first 30 postoperative days as a consequence of their primary pathology. Conclusions: Thoracoscopy was confirmed to be an effective diagnostic and therapeutic alternative for the treatment of mediastinal disorders.

Keywords: Thoracoscopy, thoracic surgery, mediastinal neoplasms, mediastinal cysts.

Benign schwannoma tumors are the most common primary tumors of the posterior mediastinum. They may develop into a dumbbell-shaped involvement of the medullar channel, but rarely reach the tracheobronchial tree. The authors report a case of a 45-year-old female patient with a benign schwannoma in the posterior mediastinum, with a dumbbell-shaped lesion that traverses the intercartilaginous septa of the trachea. The tumor was resected by endoscopy in the endotracheal portion and afterwards by thoracotomy of the mediastinal tumor. Eight months after the surgery, there has been no recurrence of the lesion.

Keywords: Mediastinal neoplasms. Thoracotomy. Thoracoscopy. Mediastinal cyst.

Objective: To investigate the effects of maternal protein malnutrition during lactation on the elastic fibers in the tracheas of Wistar rat pups. Methods: At delivery, 12 male pups of two Wistar rat dams were equally divided into two groups: control, in which the dam received water and standard rat chow ad libitum during lactation; and protein-restricted (PR), in which the dam received water ad libitum and an isoenergetic PR diet (8% protein). At 21 days of age, the pups were killed and their tracheas were excised. The elastic fibers were stained with Weigert's resorcin-fuchsin (after oxidation) and evaluated under light microscopy. Morphometric determinations were performed by stereology, with the point-counting method, and expressed as volumetric densities. Results: Elastic fibers, most having a longitudinal distribution, were identified beneath the tracheal mucosa. In addition, well-defined circular layers of elastic fibers were found around the inner and outer surfaces of the cartilaginous ring. There were no differences between the groups regarding the organization and distribution of the elastic fibers. The volumetric density of the elastic fibers of the pups in the control and PR groups was 2.46 ± 0.99% and 3.25 ± 1.13%, respectively (p < 0.01). Conclusions: The volumetric density of elastic fibers appears to be greater in rat pups breastfed by dams receiving a PR diet than in those breastfed by dams receiving a normal diet.

Keywords: Trachea/growth and development; Trachea/anatomy and histology; Extracellular matrix; Airway remodeling; Malnutrition.

Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions.

Keywords: Cystic fibrosis/diagnosis; Cystic fibrosis/therapy; Cystic fibrosis/complications; Practice guideline.

Postemetic rupture of the esophagus, also known as spontaneous rupture or Boerhaave's syndrome, was first described by Herman Boerhaave in 1724. This is a severe disease that causes high mortality rates and is difficult to diagnose not only because it is rare but also because it is frequently confused with other severe clinical conditions, such as acute myocardial infarction, perforated peptic ulcer, and acute pancreatitis. Herein, we describe three cases of patients with this syndrome. Two underwent primary repair of the rupture, and one underwent esophagectomy followed by reconstruction. There was one death due to septic shock in the immediate postoperative period. The other two patients presented favorable long-term evolution.

Keywords: Esophageal diseases; Rupture, spontaneous; Mediastinitis; Esophagectomy.

Background: Lobectomy is still considered the most effective method of controlling primary lung tumors. Method: During the period from 1995 to 2000, 733 cases of non-small cell bronchial carcinoma. After clinical evaluation and surgical staging, 191 patients were submitted to surgical resection. Of those 191 surgeries, 63 were for locally advanced tumors and 128 (69 segmentectomies and 59 lobectomies) for primary tumors. Post-operative FEV1 of at least 800 ml was used as a measure of surgical success. Extended segmentectomies, in which the resection passes the intersegmental line, including the parenchyma of the adjoining segment, were used. Results: Among the 128 patients with primary tumors, there were 3 deaths and 10 patients fell out of contact. Therefore, 62 segmentectomies and 53 lobectomies were evaluated. There were 72 adenocarcinomas and 43 epidermoid carcinomas. The 5-year survival of lobectomy patients was 80% (T1N0), 72.7% (T2N0), 50% (T1N1) and 31.8% (T2N1), whereas that of segmentectomy patients was 80% (T1N0), 66.6% (T2N0), 41.1% (T1N1) and 30% (T2N1) (p > 0.05). Tumor size and enlarged interlobar lymph nodes were prognostically significant (p < 0.001), although method of resection influenced neither survival nor local or remote recurrence (p > 0.05). Conclusion: Extended segmentectomy represents an alternative treatment for primary tumors in patients with limited lung reserve.

Keywords: Lung Neoplasms, surgery. Pulmonary Surgical Procedures. Carcinoma, Non-Small-Cell Lung.

Objective: Evaluate the impact of the instrument of the "Stratification by Degree of Clinical Severity and Abandonment Risk of Tuberculosis Treatment" (SRTB) on the tuberculosis outcome. Methods: This study was a pragmatic clinical trial involving patients with a confirmed diagnosis of tuberculosis treated at one of the 152 primary health care units in the city of Belo Horizonte, Brazil, between May of 2016 and April of 2017. Cluster areas for tuberculosis were identified, and the units and their respective patients were divided into intervention (use of SRTB) and nonintervention groups. Results: The total sample comprised 432 participants, 223 and 209 of whom being allocated to the nonintervention and intervention groups, respectively. The risk of treatment abandonment in the nonintervention group was significantly higher than was that in the intervention group (OR = 15.010; p < 0.001), regardless of the number of risk factors identified. Kaplan-Meier curves showed a hazard ratio of 0.0753 (p < 0.001). Conclusions: The SRTB instrument was effective in reducing abandonment of tuberculosis treatment, regardless of the number of risk factors for that. This instrument is rapid and easy to use, and can be adapted to different realities. Its application showed characteristics predisposing to a non-adherence to the treatment and established bases to mitigate its impact.

Keywords: Tuberculosis; Patient compliance; Risk factors; Treatment adherence.

Saber-sheath trachea constitutes a morphologic-functional disorder of the intrathoracic segment, characterized by an abnormal configuration of the tracheal cartilages, where the coronal diameter represents less than 2/3 of the sagittal, with narrowing of the front bow and collapse of the side wall during coughing and stressed expiration. Just as it happens in the case of semilunar configuration of the thacheal expiratory collapse syndrome, it is more common in patients with COPD, when the clinicradiologic appearance usually masks the seriousness of the functional disorder. The authors demonstrate the clinic-tomographic-endoscopic-functional correlation in the case of a 43-year-old female patient suffering from severe chronic obstructive respiratory failure whose clinic-radiologic condition masked the seriousness of the dysfunction, emphasizing the need for systematic investigation of the tracheal dynamics in this type of case.

Objective: Cystic fibrosis (CF) is a chronic multisystemic hereditary disease for which a multidisciplinary approach must be taken. The objective of this study was to show the evolution of a group of patients with CF after the implementation of multidisciplinary treatment. Methods: A retrospective study involving 19 patients (6-29 years of age) under clinical follow-up treatment at the University of São Paulo at Ribeirão Preto School of Medicine Hospital das Clínicas, located in the city of Ribeirão Preto, Brazil. The patients were divided into two groups: 6-12(6-12 years of age) and 13+ (> 12 years of age). We collected data regarding body mass index (BMI), Zscore, Shwachman score (SS), number of exacerbations/year, chronic colonization by Pseudomonas aeruginosa, and spirometric measurements (FVC, FEV1, FEV1%, and FEF25-75%). Data were collected at two different time points (before and after the implementation of the multidisciplinary treatment) and were analyzed with the Wilcoxon signed rank test. Results: The median age at the onset of symptoms was 10 months. In the 6-12 group, only BMI and FVC increased significantly. Although the other spirometric values increased, the differences were not significant. In the 13+ group, there were no significant differences between the two time points. There was a borderline significant decrease in SS and less than significant decreases in the spirometric measurements. However, the number of patients with alterations in volumes and flows decreased in both groups. Conclusions: Although our patient sample was small, the lack of changes in the spirometric parameters might reflect clinical and functional stability. In all of the patients evaluated, clinical, functional, and nutritional parameters remained stable throughout the study period. The implementation of a multidisciplinary approach might have contributed to this result.

Keywords: Cystic fibrosis; Spirometry; Body mass index; Physical therapy modalities.

Incidence of metastatic disease to the hypothalamic-pituitary area is low (1 to 6% in most studies), the lung and the prostate being the most common primary sites in men. Clinical presentation as panhypopituitarism is rare (0.2 to 0.8%) in patients with bronchogenic carcinoma. The authors describe a case of a man with diagnosis of squamous cell carcinoma of the lung that, during the clinical course of the disease, presented clinical and laboratorial evidence of panhypopituitarism, with an expansive sellar and supra-sellar lesion image in the CT scan of the brain.

Keywords: Panhypopituitarism. Bronchogenic carcinoma. Hypothalamic-pituitary metastases.

Objective: To analyze the locations of lung carcinomas in relation to patient gender and smoking status. Methods: In order to test the hypothesis that lung carcinoma location (upper or lower lobe; left or right side) is correlated with smoking status and gender, we conducted a retrospective study of 697 patients with bronchogenic carcinoma treated at the Pulmonology-Oncology Outpatient Clinic of the Federal University of São Paulo. Results: We found that the bronchogenic carcinomas occurring in smokers were more frequently located in the upper lobes, whereas those occurring in nonsmokers were more frequently located in the lower lobes. In women, the neoplasms were more often seen in the lower lobes, especially in nonsmokers. Based on the available data, there were no differences in terms of the side affected (left or right). Conclusion: Overall, bronchogenic carcinomas are predominantly found in the upper lobes. However, in nonsmokers, they occur more frequently in the lower lobes. In females, bronchogenic carcinomas present a tendency to occur more often in the lower lobes.

Keywords: Lung neoplasms; Sex factors; Smoking

Objective: To assess the quality of life (QoL) of patients with cystic fibrosis (CF) followed at a university referral center for CF. Methods: A cross-sectional study involving application of the Cystic Fibrosis Questionnaire (CFQ) and Shwachman score in CF patients between April of 2008 and June of 2009. Results: The sample consisted of 75 patients. The mean age was 12.5 ± 5.1 years (range, 6.1-26.4 years). The patients were divided into three groups by age in years: group I (< 12), II (12-14), and III (≥ 14). The highest and lowest CFQ scores were for the nutrition domain in group III (89.3 ± 16.2) and the social domain in group II (59.5 ± 22.3), respectively. Groups I and III differed significantly regarding the treatment domain (p = 0.001). Regarding Shwachman scores, there were significant differences between patients scoring ≤ 70 and those scoring > 70 in the social (group I; p = 0.045), respiratory (group II; p = 0.053), and digestive (p = 0.042) domains. In group III, severity did not correlate with QoL. In groups I and II, patients with an FEV1 < 80% of predicted did not differ from other patients for any CFQ domain. However, in group III, values for the following domains were significantly lower in patients with an FEV1 < 80%: physical (p = 0.012); body image (p = 0.031); respiratory (p = 0.023), emotional (p = 0.041); and social role (p = 0.024). Conclusions: It is important to assess QoL in CF patients, because it can improve treatment compliance.

Keywords: Cystic fibrosis; Quality of life; Questionnaires.

The synchronous presentation of pulmonary and hepatic nodules in a patient with previously resected bronchogenic carcinoma raises suspicion of recurrence and mandates restaging. We present the case of a 71-year-old male with a history of lobectomy with pericardial resection and mediastinal lymphadenectomy (T3N0M0). At five years after the operation, he presented with a new pulmonary lesion. Restaging detected a synchronous nodule in the liver. Despite the strong suspicion of tumor recurrence, further investigation with a percutaneous liver biopsy revealed hepatocellular carcinoma. In order to investigate the etiology of the pulmonary lesion (hypotheses of recurrent bronchial cancer and of metastatic hepatocellular carcinoma), an open lung biopsy was performed, which revealed chronic inflammatory tissue with foci of anthracosis and dystrophic calcification. The patient was submitted to a non-anatomic resection of the liver lesion. The postoperative course was uneventful, and the patient was discharged on postoperative day 10. This report highlights the relevance of the histopathological diagnosis in patients with a history of bronchogenic carcinoma and suspicion of tumor recurrence. Differential diagnoses and the treatment administered are discussed.

Keywords: Carcinoma, bronchogenic; Neoplasm metastasis; Carcinoma, hepatocellular.