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Primary tracheal adenoid cystic carcinoma (TACC) is a rare malignancy without an established treatment. Central airway obstruction due to TACC often decreases the quality of life and has life-threatening consequences. A 19-year-old man with unresectable TACC and central airway obstruction suffered from progressive cough and dyspnea after exercise. Proton beam therapy (PBT) was selected as the preferred treatment over systemic anti-cancer chemotherapy for TACC. PBT led to complete remission of TACC and the almost complete disappearance of the respiratory symptoms without adverse events. PBT is a useful and safe treatment for unresectable primary TACC.

Purpose: The purpose of this study was to conduct a matched-pair analysis to assess the impact of radiotherapy (RT) on patients with malignant tracheal tumors using the surveillance, epidemiology, and end results database. Additionally, a predictive nomogram was developed for patients with malignant tracheal tumors., Methods: Propensity score matching (PSM) was used to minimize bias between the RT and no-RT groups. We utilized both univariate and multivariate Cox proportional hazards regression analyses to identify independent prognostic factors for patients and subgroups. We developed a novel nomogram and evaluated its results using the C-index., Results: A total of 648 patients between 1975 and 2019 were included, and 160 patients in RT were 1:1 propensity score-matched with no-RT. The independent prognostic factors for patients with tracheal malignant tumors were surgery, marital status, disease extension, pathology, and age. The independent risk factors for patients without surgery included RT and disease extension. The C-index confirmed that the nomogram accurately predicted the prognosis of patients with tracheal malignant tumors., Conclusions: Our findings suggest that RT may provide a survival benefit for tracheal cancer patients who did not undergo surgery. The nomogram can be a useful tool for predicting prognosis in patients with tracheal malignant tumors., (© 2023. The Author(s).)

Primary tracheal tumors are rare, constituting approximately 0.1-0.4% of malignant diseases. Squamous cell carcinoma (SCC) and adenoid cystic carcinoma (ACC) account for about two-thirds of these tumors. Despite most primary tracheal cancers being eligible for surgery and/or radiotherapy, unresectable, recurrent and metastatic tumors may require systemic treatments. Unfortunately, the poor response to available chemotherapy as well as the lack of other real therapeutic alternatives affects the quality of life and outcome of patients suffering from more advanced disease. In this condition, target therapy against driver mutations could constitute an alternative to chemotherapy, and may help in disease control. The past two decades have seen extraordinary progress in developing novel target treatment options, shifting the treatment paradigm for several cancers such as lung cancer. The improvement of knowledge regarding the genetic and biological alterations, of major primary tracheal tumors, has opened up new treatment perspectives, suggesting the possible role of biological targeted therapies for the treatment of these rare tumors. The purpose of this review is to outline the state of knowledge regarding the molecular biology, and the preliminary data on target treatments of the main primary tracheal tumors, focusing on salivary-gland-derived cancers and squamous cell carcinoma.

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 χ 2 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., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Purposes: This study aimed to evaluate the role of radiotherapy (RT) and intensity modulated radiation therapy (IMRT) in adjuvant and definitive settings of tracheal-bronchial adenoid cystic carcinoma (TACC) treatment., Materials/methods: TACC patients (n = 133) treated with surgery and/or RT curatively in our institution between January 1 st , 1984 and December 31 st , 2017 were analyzed retrospectively., Results: Among the 116 patients undergoing surgery, 50 (43.1 %) achieved complete resections and 66 (56.9 %) had positive surgical margins. For patients with positive margins, overall adjuvant RT was correlated with no significantly improved OS (10-year: 58.0 % vs. 47.9 %; P =  0.340) and a slight LRFS benefit (5-year: 81.9 % vs.75.6 %; P =  0.056), but adjuvant IMRT showed significant superiority in both OS (10-year: 82.9 % vs. 47.9 %; P =  0.031) and LRFS (5-year: 100.0 % vs. 75.6 %; P =  0.001) in comparison with no postoperative RT. Multivariate analysis also identified adjuvant IMRT as a significant favorable factor with OS (HR = 0.186, 95 %CI: 0.039-0.883; P =  0.034). For 17 patients receiving definitive RT, IMRT achieved promising 5-year OS of 88.9 % and LRFS of 64.3 %, yet no significant difference from non-IMRT group was reached (P = 0.447 and 0.706). Different therapies presented no significantly different impact on DMFS, whilst DMFS explained more of the OS variances (P < 0.001, R 2  = 0.480) than LRFS (P < 0.001, R 2  = 0.323)., Conclusion: IMRT could confer greatly improved OS and LRFS in postoperative setting for TACC patients with positive surgical margins. IMRT was also a good therapeutic option for definitive TACC with promising survival and local control., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: There is limited understanding of tracheal carcinoma (TC) because of its rarity. We examined the efficacy of radiotherapy (RT) for patients with primary TC., Methods: We analyzed the records of 32 patients with primary TC who received RT at our center between November 1996 and December 2016., Results: Thirteen patients received adjuvant RT and 18 received definitive RT. Eight patients achieved complete remission (CR) after definitive RT. Among all patients, the 5-year overall survival (OS) rate was 46.9% and the locoregional progression free survival (LRPFS) rate was 68.1%. Univariate analysis indicated the 5-year OS was better in those with adenoid cystic adenocarcinoma than squamous cell carcinoma ( P = 0.001); the 5-year LRPFS was better in patients who received surgical resection than those who did not (92.9% vs 46.4%, P = 0.013) and in patients who received postoperative RT than in those who received definitive RT (91.7% vs 50.1%, P = 0.038). A sub-group univariate analysis indicated the 5-year PFS was better for those who received at least 68 Gy of radiation (44.4% vs 13.0%, P = 0.044). Patients who achieved CR had a better 5-year PFS than those who did not (57.1% vs 10%, P = 0.006). No patients had a toxicity of grade 3 or more., Conclusions: Adjuvant and definitive RT are safe and effective treatments for TC. Patients who received dosages of 68 Gy or more and who had complete tumor regression following definitive RT seemed to have better long-term survival.

Synovial sarcoma (SS) is an uncommon malignant tumor associated with poor prognosis, and SS arising from the trachea is even rarer, with only 3 cases reported previously. We present the case of a 19-year-old man for whom imaging studies revealed a mass of soft tissue density in the lower trachea. An en bloc trachea segmental resection and postoperative proton beam therapy were performed. Diagnosis was confirmed by the SS18-SSX gene rearrangement. The patient was alive without tumor recurrence for 18 months. This case report presents tracheal SS successfully treated with surgery and postoperative proton therapy., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We present a 28-year-old man with an early onset of recurrent respiratory papillomatosis at 1 year. The patient had undergone 31 operations over a period of 7 years. After the diagnosis of tracheal papillomatosis, he received a four-time treatment of T-tube insertion combined with laser fulguration. During the last operation, pathologic findings showed moderate dysplasia with malignancy potential. Conformal radiotherapy was then given at 5000 cGY, targeting the tracheal tumor bed. The patient experienced complete remission with no complications. His condition has lasted for 20 years, and has continued up through the time of this report., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Primary malignant tumors of the trachea are very rare with the incidence of less than two per million people per year, and only ten percent of them are adenoid cystic carcinomas. Eighty percent of all tracheal tumors are malignant. Diagnosis is usually late because the symptoms mimic other conditions such as asthma. Clinical picture may sometimes be dramatic when airway is almost closed and emergency recanalization is necessary. Diagnosis is made by chest computed tomography scan or magnetic resonance imaging. Definitive treatment is surgical resection alone or followed by radiation therapy or radiation therapy alone. Radical resection is only accomplished in about half of all cases because of the submucosal tumor growth and limited length of tracheal resection. The role of adjuvant radiation therapy in negative resection margin cases is not clear but all patients with positive resection margin benefit from radiation therapy. We present a case of a 43-year-old patient with primary adenoid cystic carcinoma of distal trachea treated by emergency bronchoscopic recanalization and resection of the tracheal tumor with end-to-end anastomosis.

Objective(s): To identify predictors for receiving adjuvant radiation therapy (RT) and investigate the impact of adjuvant RT on survival for patients with resected primary tracheal carcinoma (PTC)., Methods: The National Cancer database was queried for patients with PTC diagnosed from 2004 to 2014 undergoing resection. Patients who died within 30 days of resection were excluded to minimize immortal time bias. Kaplan-Meier methods, Cox regression modeling and propensity score weighted (PSW) log-rank tests were considered to assess the relationship between adjuvant RT and overall survival (OS). Logistic regression was performed to identify predictors associated with receiving adjuvant RT., Results: A total of 549 patients were identified with 300 patients (55%) receiving adjuvant RT. Squamous cell carcinoma (SCC) was the most common histology with 234 patients (43%). Adenoid cystic carcinoma (ACC) was second most frequent with 180 patients (33%). Adjuvant RT was not associated with OS by multivariable Cox analysis or PSW log-rank test (P values > 0.05). Patients with positive surgical margins (odds ratio (OR) 1.80, confidence interval (CI) 1.06-3.07) were more likely to receive adjuvant RT than those with negative surgical margins. Patients with ACC (OR 6.53, CI 3.57-11.95) were more likely to receive adjuvant RT compared with SCC., Conclusions: Adjuvant RT was not significantly associated with OS for patients with resected PTC in this analysis. Surgical margin status and tumor histology were associated with receiving adjuvant RT. Further investigations including prospective registry studies capturing radiation technique and treatment volumes are needed to better define which patients with resected PTC may benefit from adjuvant RT., (© The Author(s) 2019. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: To evaluate the role of radiotherapy (RT) as an adjuvant or definitive treatment in primary tracheal adenoid cystic carcinoma (ACC) for local tumor control and survival., Methods: A retrospective chart review was performed in 22 patients treated with adjuvant or definitive RT for primary tracheal ACC at a single center between November 1994 and December 2008., Results: Thirteen and 9 patients received adjuvant and definitive RT, respectively. Microscopic residual disease after surgery was pathologically reported in 11 patients. The median RT dose was 59.4 Gy for adjuvant and 74.4 Gy for definitive RT. The overall response rate for definitive RT was 77.8%. Six patients in the definitive RT group exhibited local progression (LP), whereas 14 patients in both groups exhibited distant metastasis. The most common recurrence site in cases of treatment failure was the lung parenchyma. The median follow-up duration was 123 months, and the 10-year overall survival (OS) rate was 54.2%. Although LP was the most common cause of death (4 patients), two-thirds of the patients treated with definitive RT lived for >5 years. The 5-year and 10-year LP-free survival (LPFS) rates in the definitive RT group were 66.7 and 26.7%, respectively. Patients with higher RT dose by brachytherapy boost had good 5-year OS, 83.3%, and showed no local progression till 5-years. Most of the RT-induced side-effects were mild and tolerable, but 2 patients died of tracheal stenosis without any tumor recurrence., Conclusions: Adjuvant RT may be suitable for controlling microscopic residual disease, whereas definitive RT may yield appropriate long-term survival in >50% patients with unresectable tracheal ACC. Dose escalation should be considered to warrant long-term survival in definitive RT.

Purpose: Tracheal and paratracheal malignancies present challenges in radiotherapeutic management due to their proximity to sensitive structures, central location, and because of the potential for catastrophic radiation induced fistula. The use of external beam radiation therapy and intraluminal brachytherapy has a limited number of reports in the literature. We have searched for a technique for treating tracheal and paratracheal malignancies allowing application of a high dose of radiation while minimizing dose to normal tissues. Our group has considerable experience in the use of percutaneous CT-guided radioisotope seed implants for intrathoracic malignancies. We have previously reported our technique for percutaneous CT-fluoroscopic-guided radioisotope seed implants in the management of thoracic malignancies. We have now treated several tracheal and paratracheal malignancies with our technique and report our results here. This is to our knowledge the first report of treatment of tracheal malignancy with CT-guided permanent seed implant., Methods and Materials: Three patients with tracheal adenoid cystic carcinoma were implanted with 103 Pd under CT-fluoroscopic guidance utilizing percutaneous approach., Results: All patients tolerated the procedure well, and at 9-month average followup, all show disease regression, symptom improvement, and no sign of toxicity., Conclusions: We believe this to be the first published series on CT-directed permanent seed brachytherapy for tracheal malignancies. Review of PubMed literature to 1990 discloses no prior writings on the use of permanent seed implantation for tracheal cancers. Tracheal malignancies provide a vexing radiation therapy challenge to stay within the therapeutic window. CT-directed permanent seed brachytherapy allows a high dose to be delivered to the tumor with a rapid falloff to the surrounding tissues. Short-term results from seed implant are excellent. Our 3 patients responded well in the short term to permanent seed brachytherapy with no chronic side effects and with reduction or relief of cough and pain. CT-fluoro-guided permanent seed implantation is an effective and low morbidity treatment for tracheal malignancies. Long-term followup is needed to further elucidate durability of response and toxicity., (Copyright © 2017 American Brachytherapy Society. Published by Elsevier Inc. All rights reserved.)

A 47-year old woman presented with large cell carcinoma with extensive lengthwise and circumferential invasion of the lower trachea. End-to-end anastomosis by suture lines alone may be impossible and even harmful, following tumour resection with such extensive tracheal involvement. Thus, we performed a successful tracheal reconstruction with bronchoplastic closure without complications or recurrence at 12-month follow-up. This case highlights the use of this technique for the closure of massive airway defects., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Tracheal tumors are rare. They are usually unresectable and treated primarily with external beam radiation. The use of palliative endotracheal brachytherapy (ETBT) alone in treating patients with tracheal tumors has not been reported., Methods: Using a prospective database, demographic, treatment, and outcome data of patients with tracheal tumors treated palliatively with ETBT from 2006 to 2014 were analyzed. Tumor and symptom responses were evaluated based on response evaluation criteria in solid tumors criteria. Survival, in-field disease control, symptom response, and duration of symptom responses were evaluated using descriptive analyses., Results: Sixteen ETBT (median, 2) treatments were delivered to 8 patients. Median age was 63.4 years old. Common symptoms were hemoptysis, cough, and dyspnea. Tracheal lengths of 3.5-11 cm were treated with 5-7 Gy/fraction, using 1-3 fractions. The mean overall survival was 5 months and symptom-free survival was 6.8 months, respectively. After ETBT, 88% of patients experienced symptomatic improvement (hemoptysis [n = 3/3], cough [n = 6/7], and dyspnea [n = 4/4]). One patient developed Grade 1 stenosis that did not require intervention., Conclusions: This is among the largest series of tracheal tumors treated palliatively with ETBT alone. ETBT provided effective palliation with symptom improvement and minimal toxicity., (Copyright © 2015 American Brachytherapy Society. Published by Elsevier Inc. All rights reserved.)

Background: Central airway obstruction (CAO) may be caused by various etiologies. However, conventional chest X-rays are rarely diagnostic for patients with CAO., Case Presentation: We here described a 64-year-old asymptomatic female with tracheal mucosa-associated lymphoid tissue lymphoma discovered on spirometric findings during a complete physical examination. The plateau of forced expiratory flow was consistent with CAO. A decreased peak expiratory flow rate was noted at least 3 years before the diagnosis, and was attributed to an insufficient effort by the patient. Impulse oscillometric measurements, which were taken during quiet breathing and were effort-independent, suggested elevated respiratory resistance. These abnormalities completely disappeared after radiation therapy., Conclusion: The addition of impulse oscillometry to spirometry may be useful for screening CAO in routine health examinations.

An 84-year-old man presented with a 2-month history of intermittent stridor and worsening difficulty in breathing. Chest X-ray and flexible nasendoscopy were unremarkable but following further deterioration a CT scan revealed an obstructing lesion in the distal trachea. Bronchoscopy revealed an infiltrative tumour arising 3 cm above the carina causing 90% obstruction. The mass was biopsied and surgically debrided to leave a patent airway. Histological analysis revealed a diagnosis of adenoid cystic carcinoma. Transthoracic surgical resection was unsuccessful and the patient continues to be effectively managed with periodic bronchoscopic debulking and radiotherapy. This case highlights the diagnostic and therapeutic dilemmas posed by distal tracheal lesions and the need for specialist input for effective management., (2015 BMJ Publishing Group Ltd.)

There are summarized the foreign and domestic references of recent years devoted to methodology and the efficiency of the use of intraluminal high-dose radiation brachytherapy in patients with lesions of the central bronchi and trachea caused by primary and metastatic malignant tumors. It is presented own experience of applying this method in 207 patients. It is showed that in some patients to ensure the delivery of the radiation source to the area of interest it is advisable to perform firstly endotraheobronhial surgery with recanalization of the lumen of the respiratory pathways. The best is the use of intraluminal brachytherapy with high dose radiation. Palliative intraluminal irradiation of inoperable patients allowed achieving a good immediate results (65-95%), a significant reduction of the main symptoms--hemoptoe (87-95%), dyspnea (75-90%), obstructive pneumonia phenomena (50-85%), and significantly increasing survival median from 1-3 to 9-14 months. Following performance of chemoradiotherapy permitted increasing the survival median up to 15-20 months. The number of complications of intraluminal high-dose radiation brachytherapy was small, usually--pulmonary hemorrhage (2-7%) more likely developing when using large fractions--more than 10 g for 1 session.

Background: Metallic airway stents are often used in the management of central airway malignancies. The presence of a metallic foreign body may affect radiation dose in tissue. We studied the effect of a metallic airway stent on radiation dose delivery in a phantom and an in vivo porcine model., Methods: A metallic tracheal stent was fitted onto a support in a water phantom. Point dosimeters were positioned in the phantom around the support and the stent. Irradiation was then performed on a linear accelerator with and without the stent. Metallic tracheal stents were deployed in the trachea of three pigs. Dosimeters were implanted in the tissues near (Group 1) and away (Group 2) from the stent. The pigs were then irradiated, and the dose perturbation factor was calculated by comparing the actual dose detected by the dosimeters versus the planned dose., Results: The difference in the dose detected by the dosimeters and the planned dose ranged from 1.8% to 6.1% for the phantom with the stent and 0%-5.3% for the phantom without the stent. These values were largely within the manufacturer's specified error of 5%. No significant difference was observed in the dose perturbation factor for Group 1 and Group 2 dosimeters (0.836 ± 0.058 versus 0.877 ± 0.088, P = 0.220) in all the three pigs., Conclusions: Metallic airway stents do not significantly affect radiation dose in the airway and surrounding tissues in a phantom and porcine model. Radiation treatment planning systems can account for the presence of the stent. External beam radiation can be delivered without concern for significant dose perturbation., (Copyright © 2014. Published by Elsevier Inc.)

A patient identified with tracheal cancer benefits most from evaluation by an experienced center and an extensive effort to assess the possibility of a complete surgical resection as the most efficient treatment option for cure. Localized, nonoperable disease may still be controlled by combined modality using chemotherapy and concurrent radiation., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Primitive tumors of the trachea are rare, accounting for 0.1% of the airway tumors. Cystic adenoid carcinoma (or cylindroma) represents the second most frequent type of tracheal cancers. Histologically speaking, this tumor type is divided in three patterns: cribriform, tubular and solid; it presents a slow growth, perineural invasion and potential local recurrence and metastasis. We presented herein the case of a 56-year-old female suffering from a cystic adenoid carcinoma of the low trachea. She has been treated by carinal resection with negative airway margin and complete reconstruction, with the help of an extra corporeal membrane oxygenation (ECMO)., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Objective: The purpose of this study was to evaluate the role of photodynamic therapy (PDT) in primary tracheal carcinomas., Methods: Data were obtained from patients treated with Photofrin(®) PDT for primary tracheal carcinoma at the Ohio State University. Demographic data as well as survival and response were collected., Results: Ten patients 47-79 years of age with primary tracheal carcinoma (three adenoid cystic, seven squamous histology) were treated with PDT. Treatment was part of curative-intent therapy in three patients, one of whom underwent surgery. The other seven patients received palliative PDT. Five patients received sequential radiation and two received concurrent chemotherapy. All 10 patients had improvement in obstructive symptoms within 1 month. Eight patients had objective response by bronchoscopy, and one patient had stable disease. Treatment was well tolerated. One patient developed a tracheal stricture that was successfully treated with stent placement., Conclusions: PDT is safe and provides effective palliation of obstructive symptoms in patients with primary tracheal carcinoma. PDT has a potential role in both the curative and the palliative setting.

Purpose: To conduct a matched pair analysis assessing the impact of radiotherapy (RT) in patients with resectable and unresectable primary malignant tracheal tumors using Surveillance, Epidemiology and End Results (SEER) database., Patients and Methods: The SEER registry was used to identify every patient (or "case") who received RT between 1988 and 2007 for primary malignant tracheal tumors, and to search for corresponding "controls" (not treated with RT), with the same prognostic and treatment factors (surgery on the trachea, disease extension, histology, and gender). Overall survival (OS) was calculated with the Kaplan-Meier methods. Results of OS and cumulative incidence of death from tracheal cancer in the cases and controls, and in various subsets, were compared using log-rank and Gray's tests., Results: Two hundred fifty-eight patients who received RT were identified, and 78 of these had appropriate matched controls identified, forming the basis of this analysis. In the 78 (+RT) cases, the median follow-up was 60 months (range, 10-192) in the survivors vs. 55 months (range, 2-187) in the controls (no-RT group). Patients in RT group had significantly better OS, and a lower cumulative incidence of death from tracheal cancer than no-RT patients (p < 0.05). Treatment with radiation was associated with improved survival in patients with squamous cell histology [p < 0.0001], regional disease extension [p = 0.030], or those that did not undergo resection [p = 0.038]. There were four deaths in RT group and three in no-RT group attributed to cardiac and respiratory causes., Conclusion: Our data suggest a survival benefit for the use of RT broadly for all patients with tracheal cancer. Nevertheless, the retrospective nature of this observational study limits its interpretation., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objective: We report a case of rhabdomyosarcoma of the trachea in a 14-month-old child, and we present the first reported use of proton beam therapy for this tumour., Case Report: A 14-month-old girl presented acutely with a seven-day history of biphasic stridor. Emergency endoscopic debulking of a posterior tracheal mass was undertaken. Histological examination revealed an embryonal rhabdomyosarcoma with anaplasia. Multimodality therapy with surgery and chemotherapy was administered in the UK, and proton beam therapy in the USA., Conclusion: Only three cases of rhabdomyosarcoma of the trachea have previously been reported in the world literature. This is the first reported case of treatment of this tumour with proton beam therapy. Compared with conventional radiotherapy, proton beam therapy may confer improved long-term outcome in children, with benefits including reduced irradiation of the spinal cord.

Adenoid cystic carcinoma is a rare malignancy that usually originates in the salivary glands of the head and neck but has rarely been known to originate in the trachea. This histology has a predilection for perineural invasion and a tendency for both local and distant recurrences. While surgical resection is the mainstay of treatment of tracheal adenoid cystic carcinoma, tumor size, location, and patient comorbidities may preclude surgery, and the optimal nonsurgical management remains undefined. In the absence of locoregional lymph node metastases, we recommend highly conformal radiotherapy alone to a dose of 80 Gy. We report on two patients with unresectable disease who were treated with definitive radiotherapy: one using conventional photons and one treated with a combination of photon and proton beams. Both patients were treated to a dose of 80 Gy with acceptable toxicities and objective clinical and radiographic response. The patient treated with conventional photons has no evidence of recurrent disease at 5 years; the patient treated with protons has continued evidence of response without evidence of disease recurrence 11 months after treatment.

Primary malignant lymphoma of the trachea is rare. We report a case of a 60-year-old woman with a mucosa-associated lymphoid tissue lymphoma of the trachea and review the existing literature. This case highlights the importance of including intraluminal lesions in the differential diagnosis when a variable degree of obstruction is seen on pulmonary function testing.

Purpose: Delivering adequate dose to tracheal mucosa recurrence after multiple prior courses of surgery and radiation presented a challenge for radiation delivery. Tumor bed location and size, combined with previous doses to surrounding areas, complicated the use of external beam therapy with either photons or electrons. High-dose-rate (HDR) brachytherapy was explored to provide sufficient dose coverage., Methods and Materials: A 45-year-old gentleman presented with recurrent head and neck cancer. After undergoing additional excision of gross tumor in the tracheal region, radiation was recommended to improve local control. The region of residual tumor was confined to a small superficial lesion at the posterior-superior aspect of the trachea, involving mucosa located along the bend of the trachea, immediately deep to the stoma. External beam treatment was discussed but was not recommended based on recurrence location in the prior radiation field and patient's flexed chin position. HDR technique with a custom applicator was preferred., Results: A three-dimensional HDR plan based on computed tomography used a single catheter optimized to cover gross tumor volume as delineated by physician. Prescribed dose was 5 Gy/fraction for six fractions (two fractions/wk). The applicator position was verified daily with computed tomography and physician setup approval before treatment. The patient was positioned on a wing board to allow access to the stoma. HDR brachytherapy was well tolerated., Conclusions: Intraluminal HDR brachytherapy is a viable option for providing dose to region inside tracheal stoma. Advantages over photon and electron beam therapy include reduced dose to surrounding tissues previously irradiated, skin dose, and reproducibility of treatment delivery., (Copyright © 2011 American Brachytherapy Society. Published by Elsevier Inc. All rights reserved.)

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.

Primary anaplastic large cell lymphoma of the trachea is a rare tumor. Common complaints are dyspnea and cough that could mimic a partially refractory asthma in some cases. We report a 16-year-old female with an anaplastic large cell lymphoma (null cell type) in which tracheal involvement was presented with life-threatening airway obstruction and subcutaneous emphysema. After debulking the tumor by endobronchial curettage, the patient was treated with chemotherapy followed by local radiotherapy. She had no evidence of local or distant recurrence after 25 months. Primary anaplastic large cell lymphoma of the trachea is a rare life-threatening disease. Nevertheless, this condition has a good prognosis if diagnosed immediately and treated with chemotherapy and radiotherapy.

With the commercial introduction of the Varian RapidArc, a new modality for treatment planning and delivery, the need has arisen for consistent and efficient techniques for performing patient-specific quality assurance (QA) tests. In this paper we present our methodology for a RapidArc treatment plan QA procedure. For our measurements we used a 2D diode array (MapCHECK) embedded at 5 cm water equivalent depth in MapPHAN 5 phantom and an Exradin A16 ion chamber placed in six different positions in a cylindrical homogeneous phantom (QUASAR). We also checked the MUs for the RapidArc plans by using independent software (RadCalc). The agreement between Eclipse calculations and MapCHECK/MapPHAN5 measurements was evaluated using both absolute distance-to-agreement (DTA) and gamma index with 10% dose threshold (TH), 3% dose difference (DD), and 3 mm DTA. The average agreement was 94.4% for the DTA approach and 96.3% for the gamma index approach. In high-dose areas, the discrepancy between calculations and ion chamber measurements using the QUASAR phantom was within 4.5% for prostate cases. For the RadCalc calculations, we used the average SSD along the arc; however, for some patients the agreement for the MUs obtained with RadCalc versus Eclipse was inadequate (discrepancy > 5%). In these cases, the plan was divided into partial arc plans so that RadCalc could perform a better estimation of the MUs. The discrepancy was further reduced to within ~4% using this approach. Regardless of the variation in prescribed dose and location of the treated areas, we obtained very good results for all patients studied in this paper.

Tracheal chondrosarcoma are rare diseases, with only 15 cases previously described in the literature between 1959 and 2008. Here we present a rare case of tracheal chondrosarcoma and a review of the literature. Our patient, a 72-year-old man, had progressive throat pain for 2 years before diagnosis. Clinical and imaging investigation revealed a giant tracheal mass that was partially debulked by laser for symptomatic relief. Histologically, the mass was characterized as a low-grade tracheal chondrosarcoma. The patient underwent external-beam radiotherapy (EBRT) and received 60 Gy. At the time this report was written, 7 years after the end of the treatment, the patient was alive and asymptomatic without evidence of locoregional disease. This case and some described in the literature demonstrate the value of EBRT as a single treatment modality in achieving local control. More experience is required to establish the definitive role of radiotherapy in low-grade tracheal chondrosarcoma.

Basaloid squamous cell carcinoma is an uncommon variant of squamous cell carcinoma of the trachea. We describe the case of an unresectable basaloid squamous cell carcinoma of the trachea treated with concurrent chemoradiotherapy up to a dose of 60 Gy in 33 fractions with weekly paclitaxel and carboplatin. The pathological recognition of basaloid squamous cell carcinoma and its distinction from adenoid cystic carcinoma of the trachea is important for its management. Combining systemic chemotherapy with locoregional radiation is a logical approach to treatment, especially for the basaloid squamous cell carcinoma of the trachea, given its tendency to metastasize early after definitive therapy.

Objective: We investigate the influence of tumour and resection characteristics on survival in adenoid cystic carcinoma (ACC) of the trachea., Methods: A retrospective study of 12 laryngotracheal, 58 tracheal and 38 carinal resections for primary ACC in 108 consecutive operative survivors between 1962 and 2007 was conducted. Postoperative radiotherapy was administered to 82% of patients (89/108). Depth of invasion, extramural extent, organ invasion, perineural growth, margin status and lymph node involvement were described., Results: The tumour was intramural in 15% (16/108), extramural in 85% (92/108) and invaded adjacent organs in 20% (22/108). Airway margins were grossly positive in 9 (8%), microscopically positive in 59 (55%) and negative in 40 (37%) of 108 resections. Adventitial (radial) margins of transmural sections were grossly positive in 3 (3%), microscopically positive in 95 (88%) and negative in 10 (9%) cases. Perineural growth was present in 37 (34%) and absent in 12 (11%); it was not observed in 59 (55%) cases. Lymph nodes were positive in 16 (15%) and negative in 45 (42%) cases; it was not sampled in 47 (44%) cases. Median overall survival (OS) and disease-free survival (DFS) for the entire group were 17.7 and 10.2 years, respectively. OS was longer after resection with: negative airway margins (20.4 vs 13.3 years, P=0.028) and negative radial margins (21.7 vs 13.3 years, P=0.050); absence of extramural disease (21.7 vs 13.3 years, P=0.007), perineural growth (22.8 vs 7.5 years, P=0.011) or lymph node metastases (16.8 vs 6.1 years, P=0.017). DFS was longer after resection with: negative airway margins (16.6 vs 9.3, P=0.005) and absence of extramural disease (17.9 vs 9.3 years, P=0.008), perineural growth (17.9 vs 6.6 years, P=0.033) or lymph node metastases (10.2 vs 3.0 years, P=0.005)., Conclusions: After tracheal resection for ACC, limited tumour extent and complete resection are associated with longer overall and disease-free survival. Long-term survival (>10 years), however, is also observed after tracheal resection of locally advanced ACC., (Copyright 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.)

Between 1980 and 2007, five patients were pathologically diagnosed as tracheobronchial adenoid cystic carcinoma (ACC). All five patients were women aged 37-67 years. Four tumors were located in the larger airways, and one tumor was located in the peripheral lung. The following operations were done: bronchoplastic procedures in three (carinal resection with doublebarreled carinoplasty in one, sleeve right pneumonectomy in one, sleeve middle lobectomy in one), left pneumonectomy in one, and left upper lobectomy in one. Three of the five patients have survived for 172, 144, and 10 months after surgery, respectively. The best local treatment for ACC of the major airway is considered to be sleeve resection of the trachea or bronchus in an area where airway reconstruction may not be disturbed and to add postoperative irradiation when there is residual carcinoma at the stump. However, it seems controversial to recommend adjuvant radiotherapy in all patients undergoing resection.

Fistulas between the aorta and tracheobronchial tree are rare fatal complications that occur in patients treated with external and endobronchial radiation for carcinomas of the upper and lower respiratory tract. The etiopathogenesis can be multifactorial. We report the case of a patient with irradiated tracheal carcinoma who died of massive hemoptysis 25 months after completing therapy. Autopsy revealed a fistula between the trachea and arch of the aorta. Multiple colonies of cocci and bacilli were demonstrated within the media at the site of rupture and were absent in other areas of the aorta. The development of an aortotracheal fistula in this patient was most probably related to infection in an area of tracheal and vascular wall previously damaged by exposure to high doses of radiation.

Purpose: To examine the efficacy of fast neutron radiotherapy in the treatment of locally advanced adenoid cystic carcinoma (ACC) of the trachea and to compare outcomes with and without high-dose-rate (HDR) endobronchial brachytherapy boost., Methods and Materials: Between 1989 and 2005, a total of 20 patients with ACC of the trachea were treated with fast neutron radiotherapy at the University of Washington. Of these 20 patients, 19 were treated with curative intent. Neutron doses ranged from 10.7 to 19.95 Gy (median, 19.2 Gy). Six of these patients received an endobronchial brachytherapy boost using an HDR (192)Ir source (3.5 Gy x 2 fractions). Median duration of follow-up was 46 months (range, 10-121 months)., Results: The 5-year actuarial overall survival rate and median overall survival for the entire cohort were 89.4%, and 97 months, respectively. Overall survival was not statistically different among those patients receiving an endobronchial boost compared with those receiving neutron radiotherapy alone (100% vs. 68%, p = 0.36). The 5-year actuarial locoregional control rate for the entire cohort was 54.1%. The locoregional control rate was not statistically different among patients who received an endobronchial boost compared with those who received neutron radiotherapy alone (40% vs. 58%, p = 0.94). There were no cases of Grade > or =3 acute toxicity. There were 2 cases of Grade 3/4 chronic toxicity., Conclusions: Fast neutron radiotherapy is an effective treatment for locally advanced adenoid cystic carcinoma of the trachea, with acceptable treatment-related toxicity.

Lymphomatous involvement of the airway causing stridor is a rare but frightening presentation of an eminently treatable condition. We describe a 24-year-old woman with tracheal non-Hodgkin lymphoma who was initially diagnosed with asthma, but subsequently presented with near-fatal acute upper airway obstruction because of a tracheal Anaplastic Lymphoma Kinase (ALK)+ anaplastic T-cell lymphoma. The obstructing tumor was extricated by means of rigid bronchoscopy. After six cycles of Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone chemotherapy, the patient went into complete clinical remission. A high index of suspicion in patients with dyspnoea and wheeze unresponsive to bronchodilators is crucial in early diagnosis of tracheal tumors.

Primary tumors of the trachea are rare and are usually malignant in adults and benign in children. Adenoid cystic carcinoma, which is of salivary gland origin, account for about one thirds of adult primary tracheal tumors. A 49-year-old gentleman presented to us after undergoing a pneumonectomy elsewhere. Computed tomography scan of the thorax at our hospital showed a residual disease in the primary site, size of which was same as that in the preoperative scan. Because there was a compromised respiratory reserve due to pneumonectomy we decided to keep the radiation dose to the remaining lung as low as possible. We treated him by positron emission tomography-computed tomography (PET-CT) directed intensity modulated radiation therapy to a dose of 60 Gy in 30 Fractions over 6 weeks on a linear accelerator. PET helped in exact localization of the target on the planning CT. He tolerated the treatment very well. PET-CT done 1 year posttreatment showed no residual disease. Presently he is disease free with good pulmonary reserve.

Small cell carcinoma of the trachea is a rare entity and only a few cases have been described, none as a second malignant neoplasm. This is the first report of a metachronous second primary of the trachea with small cell histology in a breast cancer survivor. A 25-year-old woman was diagnosed initially with an infiltrating ductal carcinoma of the breast, and was treated with modified radical mastectomy followed by adjuvant chemo-radiotherapy. 10 years later, she presented with breathlessness and central airway obstruction. Bronchoscopy revealed an intraluminal lesion in the proximal trachea, which was reported as small cell carcinoma on biopsy. There was no evidence of loco-regional recurrence of the previously treated breast cancer. Whole-body positron emission tomography did not show any distant metastases. As it was a small cell carcinoma, she was treated with concurrent chemo-radiotherapy and remains loco-regionally controlled. Decision-making in such instances should take into account prior treatment and needs to be individualized. There is a need for increased awareness amongst primary care physicians regarding second malignant neoplasms in the long-term follow-up of breast cancer patients treated with radiation and chemotherapeutic agents that have carcinogenic potential.

Brachytherapy is a modality of treatment available for lung cancer with tracheal involvement. Correct positioning of the brachytherapy catheter is vital to the optimisation of treatment effect and reduction of complications. Normal airway anatomy and tumour location can make this positioning difficult. The current study presents the case of a 65-yr-old male with invasive tracheal squamous cell carcinoma of the anterior main carina involving the proximal left and right bronchus. The patient was successfully treated with brachytherapy using a novel modified airway stent with a traversing suture for positioning and stabilising the brachytherapy catheters and maximisation of the radiation effect. This simple yet innovative modification of readily available bronchoscopic equipment permits approximation and fixation of a brachytherapy catheter to any part of the proximal airway. Further analysis of this technique, including a prospective controlled trial, is planned.

Endobronchial obstruction associated with lung cancer represents a common and potentially life threatening complication of newly diagnosed or recurrent disease. The vast majority of patients with obstructive lesions are not curable so it is desirable to palliate these patients as quickly as possible without compromising quality of life. High dose rate brachytherapy (HDR-BT) represents a therapeutic option with several advantages over external beam radiotherapy (EBRT), particularly in previous irradiated patients. The primary objective of this retrospective analysis was to assess palliation efficacy and complication rate of repeated brachytherapy treatment in previously irradiated patients. Between July 2000 and December 2005, 270 patients with endobronchial recurrence after prior given HDR-BT and / or another treatment modality were again treated with HDR-BT. It makes 270 of 1036 patients (26 %) treated on lung cancer with HDR-BT at all in this period. Brachytherapy was delivered with a dose per fraction 8 or 10 Gy specified from the center of the source at 0,5 or 1,0 cm. The symptomatic response rate were as follows: dyspnea had a 76 % response rate, cough 77 % response rate, hemoptysis 92 % response rate, and post-obstructive pneumonia 82 % response rate, respectively. Among 270 of the patients 218 had follow up endoscopic examination (1 â 3 months after brachytherapy completion). Total response rate in this group was 80 %. Of 200 patients whose chest x-ray showed evidence of collapse or atelectasis caused by endobronchial recurrence obstruction, 146 (73 %) had evidence of re-aeration. The median duration of palliation, marked by symptoms or a chest x-ray that worsened was five months, the range varying from 2 to 14 months. We have noticed superficial mucosal necrosis in 166 of patients (61,5 %) and broncho-esophageal fistula in 6 of patients (2,2 %). Repeated HDR-BT effectively relieves the symptoms of endobronchial obstruction due to recurrent lung cancer and can be given safely as an outpatient procedure. Future studies should aim to determine the maximum tolerated dose and appropriate patient selection.

Adenoid cystic carcinoma, also called cylindroma, is the second most common histological type of tracheal malignancy but represents 1% of all respiratory tract cancers. We report a case of a 59-year-old patient submitted to an incomplete resection of the trachea and subsequently treated with adjuvant tomotherapy. There have been no reports in the literature regarding intensity-modulated radiation therapy with linac or tomotherapy systems in adenoid cystic carcinoma of the trachea. The present clinical case demonstrates the feasibility of adjuvant intensity-modulated radiation therapy techniques for optimizing the dose coverage of the tumor bed while sparing surrounding normal tissues. A dosimetric comparison between the tomotherapy plan and a 3-dimensional conformal radiotherapy plan is also reported. We demonstrate that tomotherapy permits an increase in the dose per fraction without important acute adverse effects. At 24 months' follow-up, our patient shows no evidence of disease with negative histological findings.