Treatment outcomes of patients with primary tracheal tumors - analysis of a large retrospective series.

OBJECTIVE: Primary tracheal tumors are very rare and their management is not definitely established. Due to its rarity, providing patient care in terms of optimal management poses a considerable challenge. The purpose of this study was to investigate treatment outcomes in patients with these rare tumors. METHODS: We carried out a retrospective analysis of 89 patients with primary tracheal tumors treated at the Maria Sklodowska-Curie National Research Institute of Oncology in Warsaw, Poland, over sixteen years. The study assessed patient demographics, tumor characteristics and treatment. Different treatment options were compared in terms of overall survival, disease-free survival, and progression-free survival. RESULTS: A total of 89 patients were included in the study. In the group presented, 45 patients underwent primary radical treatment and 44 were qualified for palliative treatment. Surgical resection was performed in 13 patients out of radically treated patients. The 5 year OS rates in the group of patients who underwent radical treatment and in the group of patients who underwent palliative treatment were 45.9% and 2.3%, respectively. In the group of patients who underwent radical surgical treatment, the 5 year OS was 76.9% compared to 35.8% in the group of patients who underwent nonsurgical treatment. CONCLUSION: A multidisciplinary team should decide treatment options, including in-depth consideration of surgical treatment options.

Long-Term Outcome of Definitive Chemoradiotherapy and Induction Chemoradiotherapy Followed by Surgery for T4 Esophageal Cancer with Tracheobronchial Invasion.

BACKGROUND: T4 esophageal cancer (EC) that invades the trachea or bronchus often has poorer prognosis than other T4 ECs. We investigated the long-term results of definitive chemoradiotherapy (dCRT) or induction chemoradiotherapy followed by surgery (iCRT-S) in patients with T4 EC with tracheobronchial invasion (TBI). PATIENTS AND METHODS: From 2003 to 2013, 71 patients with T4 EC with TBI were treated in our institution; 58 underwent dCRT, and 13 underwent iCRT-S. The long-term results associated with survival were retrospectively analyzed, and prognostic factors were examined by univariable and multivariable analysis. RESULTS: The 1-, 2-, and 5-year overall survival for all patients with T4 EC with TBI treated by dCRT or iCRT-S was 57, 29, and 19%, respectively. Multivariable analysis revealed that clinical lymph node (LN) metastasis and the treatment period were significant prognostic factors. Clinical LN positivity had significantly poorer prognosis than LN negativity. The treatment outcome in the later period was significantly better than that in the earlier period. In particular, the outcome after dCRT revealed significantly better prognosis in the later compared with the earlier period, whereas the outcome after iCRT-S did not show such a difference. With respect to treatment modality, no significant difference in survival was observed between dCRT and iCRT-S. CONCLUSIONS: Clinical LN negativity and later treatment period were significantly good prognostic factors for T4 EC with TBI. The recent improvements in dCRT outcomes may help to achieve survival comparable to that of iCRT-S.

Prognosis of primary tracheal tumor: A population-based analysis.

BACKGROUND: To elucidate the survival outcomes of tracheal tumors and to propose the potential stage of tracheal tumors. METHOD: All cases of primary tracheal malignant tumors were extracted from the Surveillance, Epidemiology, and End Results database (SEER) during 1973-2013. The overall survival was calculated using Kaplan-Meier method. Cox regression was utilized to identify the prognostic factors. RESULT: A total of 287 cases were finally included. The median age of the patients was 59 years. Male patients accounted for 56.1%. The median survival was 57 months. Patients were categorized as Extension1 to 4 (E1-4) and N0-N3. E1 group with size <4 cm had the best prognosis. While E1 >4 cm, E2 and E3 <3 cm groups had similar outcomes, which were superior to E3 >3 cm group. E4 was the worst. N0 patients had ideal prognosis, which were better than N1 and N2 patients. The 3-year survival rates of each T category were 74.7%, 57.3%, 28.1%, and 9.1%, respectively. In multivariate analysis, age, histology, tumor size, and extension were independent prognostic factors. CONCLUSION: Patients with old age, large tumor size, advanced extension or no surgery may have worse prognosis. The proposed T category of tracheal tumor incorporating tumor extension and size helped to predict survival outcomes.

Survival trends in patients with tracheal carcinoma from 1973 to 2011.

PURPOSE: The prognosis for primary tracheal cancer is dismal. We investigated whether there has been improvement in survival in tracheal cancer patients and how treatment modality affected overall and cancer-specific survival. MATERIALS AND METHODS: Using the Surveillance, Epidemiology, and End Results database, 1144 patients with tracheal cancer were identified between 1973 and 2011. Patients were stratified by age group, gender, race, tumor histology, and treatment modality. Radical surgery and survival rates based upon these stratifications were determined. Longitudinal analyses of survival and the percentage of patients undergoing surgery and radiation were conducted. RESULTS: In the final cohort, 327 tracheal cancer patients (34%) underwent radical surgery. Patients of younger age, female gender, and who presented with non-squamous cell tumors were statistically more likely to undergo surgery. Over time, utilization of radiation has declined while use of radical surgery has increased. Concomitantly, 5-year survival has increased from approximately 25% in 1973 to 30% by 2006. Those who did not have surgery were 2.50 times more likely to die of tracheal cancer (95% Confidence Interval 2.00-3.11, p<0.001) than those who did have surgery. Additionally, patients who underwent radical surgery alone (without adjuvant radiation therapy) were 50% or 19% less likely to die of tracheal cancer than those who underwent no treatment or combination therapy, respectively (both p<0.001). CONCLUSIONS: Survival in patients with tracheal cancer is improving over time. The utilization of radical surgery is increasing and confers the highest survival advantage to patients who are candidates.

[Clinical analysis of therapeutic bronchoscopy for tracheal neoplasm].

Objective: To analyze the clinical features in adults with tracheal neoplasm and to evaluate the efficacy of interventional bronchoscopic treatment. Methods: We retrospectively analyzed the clinical features of 43 adults undergoing therapeutic bronchoscopy for tracheal neoplasm diagnosed in Changhai Hospital affiliated to the Second Military Medical University from January 2004 to July 2014.The degree of stenosis, the grade of dyspnea, and Karnofsky performance status scale were evaluated before and after the last procedure. All cases were followed up for 2 years. Results: The 43 cases took (4.6±3.9) months on average to be diagnosed since initial symptom. The initial misdiagnosis rate was 41.9%(18/43), and 11 cases were mistaken for asthma (11/43). Malignant tumors were more common than benign tumors for tracheal neoplasm in adults. Squamous cell carcinoma and adenoid cystic carcinoma were the top 2 histological types. Central airway obstruction was completely or partially alleviated with significant relief of dyspnea after the procedures, and all 6 cases of tracheal benign tumors got complete alleviation (the overall response rate was 100%). The grade of dyspnea was 3.2±0.7 before and 1.5±0.8 after the procedures(t=6.63, P<0.05). The value of KPS was 63±12 before and 83±11 after the procedures(t=5.78, P<0.05). The 2-year survival rate of 6 cases of tracheal benign tumors was 100.0%, and 1 case of papillomatosis had a relapse. The 1-year survival rate and 2-year survival rate of 37 cases of tracheal malignant tumors were 59.5% and 43.2% respectively with a median survival of 13.6 months. Conclusion: Therapeutic bronchoscopic interventions provide significantly alleviation of central airway obstruction and result in improvement in shortness of breath and quality of life for tracheal neoplasm.

Carinal resection and reconstruction in thoracic malignancies.

BACKGROUND AND OBJECTIVES: The purpose of this study was to present clinical outcomes of malignant tumors involving the carina after surgery in order to establish the management guidelines. METHODS: Between 1996 and 2011, 30 patients underwent carinal resection and reconstruction for malignancy involving carina. We retrospectively analyzed their medical records. There were 22 cases of common type of NSCLC (squamous cell carcinoma/adenocarcinoma/large cell neuroendocrine carcinoma) and eight cases of carcinomas of salivary gland type (adenoid cystic carcinoma/mucoepidermoid carcinoma). RESULTS: Seventeen right sleeve pneumonectomies, two left sleeve pneumonectomies, nine carinal sleeve right upper lobectomies, and two airway resections and reconstructions without lung resection were performed. There were no in-hospital mortalities. Eleven postoperative morbidities occurred including three cases of acute respiratory distress syndrome following pneumonectomy. Late complications occurred in eight patients including three cases of anastomotic stenosis. During follow-up, 12 mortalities occurred, including 6 cancer-related mortalities. The 5-year overall survival rate (OS) and disease-free survival rate (DFS) were 66.3% and 52.9%, respectively. CONCLUSIONS: Malignant tumors involving the carina can be controlled with carinal surgery with acceptable mortality and morbidity. Patients with thoracic malignancy involving the carina should be considered as surgical candidate based on disease extent and functional status.

Primary tracheobronchial mucoepidermoid carcinoma--a retrospective study of 32 patients.

BACKGROUND: This retrospective study was designed to investigate the clinical characteristics, diagnosis, treatment and prognosis of primary tracheobronchial mucoepidermoid carcinoma (MEC). METHODS: Clinical data were retrospectively analyzed from 32 patients with pathologically confirmed primary tracheobronchial MEC between January 1990 and December 2010 at Zhejiang Cancer Hospital. The Kaplan-Meier methods were used to estimate and compare survival rates. RESULTS: There were 19 males and 13 females ranging in age from 7 to 73 years, with a median age of 28 years. Twenty-six of the 32 patients were treated with surgery alone. The other six patients were treated with surgery plus postoperative radiotherapy or chemotherapy. Six patients died during the follow-up time. The overall five-year survival rates were 81.25%, whereas the five-year survival rate of seven patients with high-grade tumors was only 28.6%. Stage I and II patients experienced better survival than Stage III and IV patients (the five-year survival rate was 100% and 43.6% respectively, P<0.001). CONCLUSIONS: Primary tracheobronchial MEC is a rare disease. Histologic grading and TNM (tumor-node-metastasis)staging are independent prognostic factors. Surgical resection is the primary treatment.

Trends in all causes and cause specific mortality attributable to ambient particulate matter pollution in China from 1990 to 2019: A secondary data analysis study.

BACKGROUND: Particularly fine particulate matter (PM2.5) has become a significant public health concern in China due to its harmful effects on human health. This study aimed to examine the trends in all causes and cause specific morality burden attributable to PM2.5 pollution in China. METHODS: We extracted data on all causes and cause specific mortality data attributable to PM2.5 exposure for the period 1990-2019 in China from the Global Burden of Disease 2019. The average annual percent change (AAPC) in age-standardized mortality rates (ASMR) and years of life lost (YLLs) due to PM2.5 exposure were calculated using the Joinpoint Regression Program. Using Pearson's correlation, we estimated association between burden trends, urban green space area, and higher education proportions. RESULTS: During the period 1990-1999, there were increases in mortality rates for All causes (1.6%, 95% CI: 1.5% to 1.8%), Diabetes mellitus (5.2%, 95% CI: 4.9% to 5.5%), Encephalitis (3.1%, 95% CI: 2.6% to 3.5%), Ischemic heart disease (3.3%, 95% CI: 3% to 3.6%), and Tracheal, bronchus and lung cancer (5%, 95% CI: 4.7% to 5.2%). In the period 2010-2019, Diabetes mellitus still showed an increase in mortality rates, but at a lower rate with an AAPC of 1.2% (95% CI: 1% to 1.4%). Tracheal bronchus and lung cancer showed a smaller increase in this period, with an AAPC of 0.5% (95% CI: 0.3% to 0.6%). In terms of YLLs, the trends appear to be similar. CONCLUSION: Our findings highlight increasing trends in disease burden attributable to PM2.5 in China, particularly for diabetes mellitus, tracheal, bronchus, and lung cancer.

A novel definition of extrathyroidal invasion for patients with papillary thyroid carcinoma for predicting prognosis.

OBJECTIVES: Extrathyroidal invasion is one of the most significant risk factors for patients with papillary thyroid carcinoma (PTC). The purpose of this study was to evaluate a novel definition of extrathyroidal invasion for patients with PTC as a method for predicting a patient's prognosis. METHODS: The prospective study was conducted for consecutive 930 patients with primary PTC who received surgery during 1993-2009. We defined only patients who had preoperative recurrent laryngeal nerve palsy or patients in whom the tumor had invaded to the mucosa of the trachea and/or esophagus as Ex3. Patients with minimal invasion were classified as Ex1, and patients with massive invasion, when we could shave off the tumors, were classified as Ex2. Patients without extrathyroidal invasion were classified as Ex0. RESULTS: Patients classified Ex3 showed significantly shorter disease-free survival (p = 0.03) and disease-specific survival (p = 0.007) than patients classified Ex2. The time to recurrence at resection sites was shorter in patients classified Ex3 than in patients classified Ex2 (p = 0.02). The time to death due to distant metastasis of patients classified Ex3 was significantly shorter than that of patients classified Ex2 (p = 0.02). Within the patients classified Ex3, disease-specific survival of patients with invasion to other nearby structures was shorter than that of patients with invasion to only recurrent laryngeal nerve (p = 0.008). CONCLUSIONS: The degree and site of invasion is an important prognostic factor for PTC. Our novel classification of extrathyroidal invasion is valuable in predicting the prognosis of PTC.

Does the effect of gender modify the relationship between deprivation and mortality?

BACKGROUND: In this study we propose improvements to the method of elaborating deprivation indexes. First, in the selection of the variables, we incorporated a wider range of both objective and subjective measures. Second, in the statistical methodology, we used a distance indicator instead of the standard aggregating method principal component analysis. Third, we propose another methodological improvement, which consists in the use of a more robust statistical method to assess the relationship between deprivation and health responses in ecological regressions. METHODS: We conducted an ecological small-area analysis based on the residents of the Metropolitan region of Barcelona in the period 1994-2007. Standardized mortality rates, stratified by sex, were studied for four mortality causes: tumor of the bronquial, lung and trachea, diabetes mellitus type II, breast cancer, and prostate cancer. Socioeconomic conditions were summarized using a deprivation index. Sixteen socio-demographic variables available in the Spanish Census of Population and Housing were included. The deprivation index was constructed by aggregating the above-mentioned variables using the distance indicator, DP2. For the estimation of the ecological regression we used hierarchical Bayesian models with some improvements. RESULTS: At greater deprivation, there is an increased risk of dying from diabetes for both sexes and of dying from lung cancer for men. On the other hand, at greater deprivation, there is a decreased risk of dying from breast cancer and lung cancer for women. We did not find a clear relationship in the case of prostate cancer (presenting an increased risk but only in the second quintile of deprivation). CONCLUSIONS: We believe our results were obtained using a more robust methodology. First off, we have built a better index that allows us to directly collect the variability of contextual variables without having to use arbitrary weights. Secondly, we have solved two major problems that are present in spatial ecological regressions, i.e. those that use spatial data and, consequently, perform a spatial adjustment in order to obtain consistent estimators.

Patterns of Care and Outcomes of Primary Adenoid Cystic Carcinoma of the Trachea.

OBJECTIVE: Primary tracheal malignancies are relatively rare cancers, representing 0.1% to 0.4% of all malignancies. Adenoid cystic carcinoma (ACC) is the second most common histology of primary tracheal malignancy, after squamous cell carcinoma. This study aims to analyze demographic characteristics and potential influencing factors on survival of tracheal ACC (TACC). METHODS: This was a retrospective cohort study utilizing the National Cancer Database (NCDB). The NCDB was queried for all cases of TACC diagnosed from 2004 to 2016 (n = 394). Kaplan-Meier (KM) and Cox proportional-hazards models were used to determine clinicopathological and treatment factors associated with survival outcomes. RESULTS: Median age of diagnosis was 56 (IQR: 44.75-66.00). Females were affected slightly more than males (53.8% vs 46.2%). The most prevalent tumor diameter range was 20 to 39 mm (34.8%) followed by greater than 40 mm in diameter (17.8%). Median overall survival (OS) was 9.72 years with a 5- and 10-year OS of 70% and 47.5%, respectively. Localized disease was not associated with a survival benefit over invasive disease (P = .388). The most common intervention was surgery combined with radiation therapy (RT) at 46.2%, followed by surgery alone (16.8%), and standalone RT (8.9%). When adjusting for confounders, surgical resection was independently associated with improved OS (HR 0.461, 95% CI 0.225-0.946). Tumor size greater than 40 mm was independently associated with worse OS (HR 2.808; 95% CI 1.096-7.194). CONCLUSION: Our data suggests that surgical resection, possibly in conjunction with radiation therapy, is associated with improved survival, and tumor larger than 40 mm are associated with worse survival.

Evaluation of Release Maneuvers After Airway Reconstruction.

BACKGROUND: Airway release (AR) maneuvers performed during airway resection to reduce anastomotic tension have not been thoroughly studied. METHODS: This study retrospectively analyzed consecutive resections for postintubation stenosis (PITS) and primary tracheal neoplasms (PTNs) at Massachusetts General Hospital (Boston, MA). Anastomotic complications were defined as stenosis, separation, necrosis, granulation tissue, and air leak. Logistic regression modeling was used to identify factors associated with AR and adverse outcome. RESULTS: From 1993 to 2019, 545 patients with PITS (375; 68.8%) and PTNs (170; 31.2%) underwent laryngotracheal, tracheal, or carinal (resections and reconstructions; 5.7% (31 of 545) were reoperations. AR was performed in 11% (60 of 545): in 3.8% of laryngotracheal resections (6 of 157; all laryngeal), in 9.8% of tracheal resections (34 of 347; laryngeal, 12, and hilar, 22), and in 49% of carinal resections (20 of 41; laryngeal, 1, and hilar, 19). Mean resected length was 3.5 cm (range, 1to- 6.3 cm) with AR and 3.0 cm (range, 0.8 to 6.5 cm) without AR (P < .01). Operative mortality was 0.7% (4 of 545); all 4 anastomoses were intact until death. Anastomotic complications were present in 5% of patients who underwent AR (3 of 60) and in 9.3% (45 of 485) of patients who did not. AR was associated with resection length of 4 cm or longer (odds ratio [OR], 6.15; 95% confidence interval [CI], 1.37 to 27.65), PTNs (OR, 7.81; 95% CI, 3.31 to 18.40), younger age (OR, 0.96; 95% CI, 0.94 to 0.98), and lung resection (OR, 6.09; 95% CI, 1.33 to 27.90). Anastomotic complications in patients with tracheal anastomoses were associated with preexisting tracheostomy (OR, 2.68; 95% CI, 1.50 to 4.80), but not release. CONCLUSIONS: Tracheal reconstruction succeeds, even when anastomotic tension requires AR. Because intraoperative assessment may underestimate tension, lowering the threshold for AR seems prudent, particularly in patients with diabetes.

[Radical radiotherapy of primary cancer of the trachea].

The paper deals with the importance of treatment of primary cancer of the trachea and, in particular, the experience of the Center in using radical therapy for inoperable squamous cell tumors and adenoid cystic carcinoma. An original system of tumor staging developed at the Center draws on international classifications (TNIVI) and methods of radiotherapy depending on tumor localization. It is shown that tracheal malignancies, especially adenoid cystic carcinomas, are characterized by relatively high radiosensitivity.

Effective Radiotherapy in Tracheobronchial Adenoid Cystic Carcinoma With Positive Surgical Margin.

BACKGROUND: The study aimed to evaluate the role of postoperative radiotherapy (PORT) in the treatment of trachea and main bronchus adenoid cystic carcinoma (ACC) with a positive surgical margin. METHODS: Patients with pathologically confirmed trachea or main bronchus ACC operated on at Shanghai Chest Hospital were enrolled. Survival, univariate, and multivariate analyses were performed. The χ2 test was applied to analyze the failure patterns among different groups (R0/0: negative margin resection without PORT; R1/0: positive margin resection without PORT; R1/1: positive margin resection with PORT). RESULTS: From January 2001 to December 2014, 77 patients were deemed eligible for the study. Pairwise comparisons showed that the overall survival rate of group R1/1 was comparable to that of group R0/0 (P = .438), and significantly longer than the rate of group R1/0 (P = .032). Additionally, the local disease-free survival rate of group R1/1 was much higher than that of group R0/0 (P = .023) and R1/0 (P = .001). Cox multivariate analysis identified the radiologic feature (P = .012) and PORT (P = .006) as significantly favorable prognostic factors for locoregional disease-free survival. By contrast, for overall survival, PORT (P = .032) was the only corresponding variable identified by univariate analysis. Furthermore, PORT significantly decreased the locoregional recurrence rate (P = .002) but not distant metastases (P > .999). CONCLUSIONS: PORT helped patients with tracheobronchial ACC and microscopic positive surgical margins to achieve a similar outcome as patients with complete resection. R0 resection may not be necessary for tracheobronchial ACC if it is difficult to be completely resected.

Clinical features, treatment and outcomes in patients with tracheal adenoid cystic carcinoma: a systematic literature review.

BACKGROUND: Primary tracheal adenoid cystic carcinoma (TACC) is rare and originates from the minor salivary gland. Biologically, TACC results in delayed presentation, and the therapeutic effects of multimodal treatment differ across individuals. This study aimed to review cases of TACC to identify clinical features, imaging modalities, treatment, and patient outcomes across follow-ups. METHODS: The PubMed, Web of Science and MEDLINE databases were searched to identify articles reporting cases of TACC. The study variables included in the analysis were patient demographics, biological characteristics, presenting symptoms, imaging modalities, treatments, follow-up times and survival outcomes. RESULTS: A total of 76 articles and 1252 cases were included in this review. The most common presenting symptom was dyspnoea (86.0%), followed by cough (58.0%). Surgery alone (40.9%), surgery with postoperative radiotherapy (36.4%) and radiotherapy alone (19.2%) were used most frequently treatments modalities. Of the 1129 cases with disease control and survival data, there was no evidence of disease in 78.7%, local recurrence was reported in 3.8%. Distant metastasis rate was 24.9% of 418 reported cases, lung (44.2%) was the most commonly involved organ. The 5, 10 years survival rate of patients treated with surgery alone and surgery with postoperative radiotherapy were 86.4%, 55.6% and 97.3%, 44.4%, respectively. CONCLUSION: TACC most common presenting symptoms were dyspnoea, cough and shortness of breath. Surgery alone and surgery with postoperative radiotherapy are predominant treatment modalities. Both seems to provide a good result in term of disease control and long-term survival rate in patients with TACC.

The Prognostic Value of 18F-Fluorodeoxyglucose PET/CT in the Initial Assessment of Primary Tracheal Malignant Tumor: A Retrospective Study.

OBJECTIVE: To investigate the potential value of 18F-fluorodeoxyglucose (FDG) PET/CT in predicting the survival of patients with primary tracheal malignant tumors. MATERIALS AND METHODS: An analysis of FDG PET/CT findings in 37 primary tracheal malignant tumor patients with a median follow-up period of 43.2 months (range, 10.8-143.2 months) was performed. Cox proportional hazards regression analyses were used to assess the associations between quantitative 18F-FDG PET/CT parameters, other clinic-pathological factors, and overall survival (OS). A risk prognosis model was established according to the independent prognostic factors identified on multivariate analysis. A survival curve determined by the Kaplan-Meier method was used to assess whether the prognosis prediction model could effectively stratify patients with different risks factors. RESULTS: The median survival time of the 37 patients with tracheal tumors was 38.0 months, with a 95% confidence interval of 10.8 to 65.2 months. The 3-year, 5-year and 10-year survival rate were 54.1%, 43.2%, and 16.2%, respectively. The metabolic tumor volume (MTV), total lesion glycolysis (TLG), maximum standardized uptake value, age, pathological type, extension categories, and lymph node stage were included in multivariate analyses. Multivariate analysis showed MTV (p = 0.011), TLG (p = 0.020), pathological type (p = 0.037), and extension categories (p = 0.038) were independent prognostic factors for OS. Additionally, assessment of the survival curve using the Kaplan-Meier method showed that our prognosis prediction model can effectively stratify patients with different risks factors (p < 0.001). CONCLUSION: This study shows that 18F-FDG PET/CT can predict the survival of patients with primary tracheal malignant tumors. Patients with an MTV > 5.19, a TLG > 16.94 on PET/CT scans, squamous cell carcinoma, and non-E1 were more likely to have a reduced OS.

Long-term Outcomes of Juvenile Onset Recurrent Respiratory Papillomatosis with Pulmonary Involvement.

OBJECTIVE: To investigate the clinical characteristics and long-term outcomes of juvenile onset recurrent respiratory papillomatosis (JORRP) with or without pulmonary involvement. METHODS: A group of patients with JORRP who had clinical course over an extended period of time (at least 5 years) in the Department of Otolaryngology Head and Neck Surgery, Beijing Tongren Hospital were included in this retrospective study. Lung/bronchus involvement was revealed by lung imaging. Data on mortality rate, frequency of surgical interventions, and age of disease onset were collected and analyzed. RESULTS: The 192 patients (107 male and 85 female) included had a median [quartiles] age of JORRP onset of 2 [1, 4] years, and median follow-up duration of 10 [7, 13] years; 17 patients (8.9%) had papilloma with bronchial and pulmonary involvement 7.0 [4.0, 12.5] years after the onset of the disease. Compared to patients without lung involvement, patients with lung involvement had a younger age of disease onset (P = .001), higher frequency of surgical interventions (P < .001), higher mortality rate (OR = 94.909), and an increased risk of tracheotomy that could not be decannulated (P < .001). They also had a younger age of disease onset, and a higher frequency of surgical interventions and mortality compared to patients with tracheotomy but free from lung involvement (P < .001). CONCLUSIONS: Children with JORRP and with pulmonary involvement have a higher average number of operations per year than those without pulmonary involvement, and pulmonary involvement indicates a higher incidence of tracheotomy that cannot be decannulated. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2277-E2283, 2021.

[Diagnosis and treatment of tracheal or bronchuotracheal adenoid cystic carcinoma].

BACKGROUND AND OBJECTIVE: Adenoid cystic carcinoma is primary bronchopulmonary carcinoma with low malignancy, and 43 patients treated in the past 50 years in our hospital were retrospectively studied. The aim of this study is to discuss the clinical symptoms, pathologic characteristic and therapeutic method of primary tracheal or bronchuotracheal adenoid cystic carcinoma. METHODS: This study summarized total 43 patients of primary tracheal or bronchus adenoid cystic carcinoma treated in our hospital from Jan. 1958 to Dec. 2007. Among them, 40 patients were treated by surgical resection, and 3 patients were treated by fiberoptic bronchoscope's interventional treatment. RESULTS: The 1-yr, 3-yr, 5-yr survival rates of the 43 patients above were 100% (41/41), 89.5% (34/38), 87.1% (27/31), respectively. CONCLUSION: Primary tracheal or bronchus adenoid cystic carcinoma are rare and low malignancy carcinoma. The clinical symptoms of them are not typical. The best treatment is early detection and taking measures of operation plus radiotherapy. The other palliative treatment is fiberoptic bronchoscope's interventional treatment.

Pediatric airway tumors: A report from the International Network of Pediatric Airway Teams (INPAT).

OBJECTIVE: Primary tracheobronchial tumors (PTTs) are rare heterogeneous lesions arising from any part of the tracheobronchial tree. Nonspecific symptoms may lead to delayed diagnosis that requires more aggressive surgical treatment. An analysis of cases collected by the International Network of Pediatric Airway Team was undertaken to ensure proper insight into the behavior and management of PTTs. METHODS: Patients <18 years of age with a histological confirmation of PTT diagnosed from 2000 to 2015 were included in this multicenter international retrospective study. Medical records, treatment modalities, and outcomes were analyzed. The patient presentation, tumor management, and clinical course were compared between malignant and benign histotypes. Clinical and surgical variables that might influence event-free survival were considered. RESULTS: Among the 78 children identified, PTTs were more likely to be malignant than benign; bronchial carcinoid tumor (n = 31; 40%) was the most common histological subtype, followed by inflammatory myofibroblastic tumor (n = 19; 25%) and mucoepidermoid carcinoma (n = 15; 19%). Regarding symptoms at presentation, wheezing (P = 0.001) and dyspnea (P = 0.03) were more often associated with benign growth, whereas hemoptysis was more frequently associated with malignancy (P = 0.042). Factors that significantly worsened event-free survival were age at diagnosis earlier than 112 months (P = 0.0035) and duration of symptoms lasting more than 2 months (P = 0.0029). CONCLUSION: The results of this international study provide important information regarding the clinical presentation, diagnostic workup, and treatment of PTTs in children, casting new light on the biological behavior of PTTs to ensure appropriate treatments. LEVEL OF EVIDENCE: NA Laryngoscope, 130:E243-E251, 2020.

Impact of Positive Margins and Radiation After Tracheal Adenoid Cystic Carcinoma Resection on Survival.

BACKGROUND: Achieving negative margins for adenoid cystic carcinoma (ACC) of the trachea can be technically difficult. This study evaluated the impact of positive margins on prognosis and tested the hypothesis that radiation improves survival in the setting of incomplete resection. METHODS: The impact of margin status and adjuvant therapy on overall survival of patients with tracheal ACC in the National Cancer Database (1998 to 2014) who underwent resection with known margin status and with no documented nodal or distant disease was evaluated using Kaplan-Meier and Cox proportional hazard analysis. RESULTS: Of 132 patients who met study criteria, 79 (59.8%) had positive margins after resection. Adjuvant radiation was given to 95 patients overall (72.0%) and to 62 of the 79 patients with positive margins (78.5%). The survival of patients with positive margins was not significantly different from that of patients with negative margins (5-year survival, 82.2% [95% confidence interval (CI), 71.3-89.3] compared with 82.0% [95% CI, 67.0-90.6], P = .97), even after multivariable adjustment (hazard ratio, 1.73; 95% CI, 0.62-4.84; P = .30). In the subset of patients with positive margins, there was no significant difference in survival between patients who did or did not receive postoperative radiation therapy (5-year survival, 82.0% [95% CI, 68.8-89.9] compared with 82.4% [95% CI, 54.7-93.9]; P = .80), even after multivariable adjustment (hazard ratio, 1.04; 95% CI, 0.21-5.25; P = .96). CONCLUSIONS: The majority of tracheal ACC resections performed in this national cohort had positive margins. Adjuvant radiation was commonly used for positive margins but was not associated with an overall survival benefit.