Search

Malignant primary tracheal tumours are rare. The most common histological subtypes are squamous cell carcinoma and adenoid cystic carcinoma. These two entities have different prognoses and growth patterns. Tracheobronchoscopy and thoracic sectional imaging are standard diagnostic tools for tumour staging and local evaluation. Complete surgical resection of the affected tracheal segment is the treatment of choice for limited disease without distant metastases. Incomplete gross tumour resection with additional irradiation is an acceptable therapeutic option for adenoid cystic carcinoma. Interventional endoscopy with tumour debulking or tracheal stenting and/or definitive mediastinal radiotherapy are treatment alternatives in either a locally advanced or palliative setting., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Background/aim: Tracheobronchial adenoid cystic carcinoma (ACC) is a rare type of malignancy. Although complete resection is standard treatment for localized ACC, treatment for unresectable ACC has not been established. It is unclear whether concurrent chemoradiotherapy (CCRT) followed by immune checkpoint inhibitor (ICI) therapy is effective for ACC., Case Report: A 49-year-old man was admitted to our hospital for the treatment of dyspnea and thickening of the bronchial wall from the tracheal carina to the left main bronchus, as observed on a CT scan. Systemic examinations and transbronchial biopsy led to a diagnosis of locally advanced ACC. Although radiotherapy and chemotherapy are not regarded as very sensitive for ACC, a favorable response was obtained with CCRT. Following CCRT, he received ICI therapy with durvalumab for 1 year. The patient has remained in a stable condition 18 months after therapy, with no recurrence., Conclusion: ICI after CCRT might be a promising treatment option for unresectable tracheobronchial ACC., (Copyright © 2024, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Background: Primary tracheal tumors are very rare and the literature on this subject is limited. The most common histological type of primary tracheal tumors is squamous cell carcinoma (SCC), followed by adenoid cystic carcinoma (ACC). Limited knowledge exists regarding the behavior and outcomes of different histological types of tracheal cancers. The present study aimed to address this gap by assessing the significance of the histological type of primary tracheal tumors based on our own data and to review the literature., 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, between 2000 and 2016. The study assessed patient demographics, tumor characteristics and treatment, with a focus on SCC, ACC, and other histological types. Different histological types were compared in terms of overall survival, disease-free survival, and progression-free survival., Results: SCC was the most frequently diagnosed histological type (56.2%), followed by ACC (21.3%). Patients with SCC were typically older (78% over 60 years), predominantly male (66%), and associated with smoking. In contrast, the ACC had a more balanced gender distribution and did not correlate with smoking. ACC displayed a significantly better prognosis, with a median overall survival of 129.4 months, compared with 9.0 months for SCC., Conclusion: Histological type plays a crucial role in the prognosis of primary tracheal tumors. ACC demonstrated a more favorable outcome compared with SCC., (© 2024 The Authors. Thoracic Cancer published by John Wiley & Sons Australia, Ltd.)

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.

Adenoid cystic carcinoma (ACC) is a rare type of malignant tracheal tumor originating from the secretory glands. Complete surgical resection is the current standard of care for tracheal ACC. However, there have been few case reports of chemoradiotherapy for unresectable tracheal ACC. We herein report a 28-year-old man with unresectable tracheal ACC who received concurrent chemoradiotherapy (CCRT) followed by maintenance therapy with durvalumab. CCRT was completed with a good response and safety, and the patient is currently receiving durvalumab as maintenance therapy. Durvalumab after CCRT can be a treatment option for patients with unresectable tracheal ACC.

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.

Objective: Tracheobronchial adenoid cystic carcinoma is a rare, slow-growing malignancy with a considerable propensity for local extension that may require complex airway resection to achieve tumor-free margins. The objective of this study was to assess whether our experience supports complex airway resection for tracheobronchial adenoid cystic carcinoma., Methods: Consecutive patients who underwent curative resection for tracheobronchial adenoid cystic carcinoma at our institution between 1970 and 2019 were included retrospectively and classified as having had complex or standard resection. Complex surgery included total tracheal replacement, associated esophageal resection, pneumonectomy, total laryngectomy with tracheal resection, and carinal resection. Standard surgery included tracheal resection, bronchoplastic resection, lobectomy, and bilobectomy. We obtained data from medical records, referring physicians, patients, relatives, and public death records., Results: Of 59 included patients, 38 had complex and 21 had standard surgery. All 4 (6.8%) patients who died postoperatively had undergone complex surgery. Postoperative morbidity was 32.2% overall and was significantly higher after complex surgery (P = .043). Overall 5- and 10-year survival rates were 81.5% and 60.2%, with no significant differences between groups (P = .31). By univariate analysis, T4 tumor and microscopically detectable tumor in the operative specimen margins and gross tumor in the operative specimen margins were associated with poorer survival (P < .05). In the subgroup with microscopically detectable tumor resection, survival was significantly better with adjuvant radiotherapy (P < .05)., Conclusions: Complex resection for extended tracheobronchial adenoid cystic carcinoma may achieve local control and satisfying long-term survival. However, this demanding procedure is associated with high postoperative morbidity and mortality rates. Because adjuvant radiotherapy improved outcomes after resection resulting in microscopically detectable tumor in the operative specimen margins, expected outcomes after resection with no detectable tumor in the margins must be compared to those after resection resulting in microscopically detectable tumor in the margins plus radiotherapy, according to the operative risk., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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.)

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.

Competing Interests: Competing interests: None declared.

Objective: Tracheobronchial mucoepidermoid carcinoma (TMEC) is an extremely rare salivary gland-type neoplasm. We aimed to explore the clinical characteristics and prognosis of TMEC and to compare them with those of another rare salivary gland-type neoplasm, tracheobronchial adenoid cystic carcinoma (TACC)., Method: We performed a retrospective review of all patients pathologically diagnosed with TMEC between 1965 and 2017 at our institution. We reviewed the patients' clinical characteristics, treatment methods and outcomes and compared the results of TMEC and TACC patients., Results: A total of 115 consecutive patients, including 107 who underwent surgery and 8 who received nonoperative therapy, were included in our study. The 1-, 2-, and 5-year survival rates were 97.89%, 94.17%, and 90.50%, respectively, in the surgical group and 83.33%, 41.67% and 0.00%, respectively, in the nonoperative group. The multivariate analysis showed that N stage was an independent prognostic factor for overall survival (OS). TMEC patients were younger, had a shorter complaint duration, had fewer symptoms, had more bronchial tumors, and were more likely to undergo surgical treatment and achieve an R0 resection (surgically treated patients) than TACC patients; furthermore, TMEC patients had a significantly better OS than TACC patients (P < 0.050)., Conclusions: TMEC has different characteristics and a better prognosis than TACC, which may reflect the different biological behaviors of these two salivary gland neoplasms. Radical treatment and close follow-up are critical for surgically treated TMEC patients with lymph node metastasis., Competing Interests: Declaration of competing interest The authors declare that they have no competing interests., (Copyright © 2020 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Objective: To explore the efficacy and safety of the covered airway stent loaded with (125)I seeds for the treatment of tracheal adenoid cystic carcinoma (TACC). Methods: We retrospectively reviewed the clinical data from 8 patients with TACC who had received placement of the covered stent loaded with (125)I seeds between December 2014 and July 2017 in the endoscopic center of the Second Affiliated Hospital of Xiamen Medical College. We compared the difference in the dyspnea index, the diameter of the airway lumen, and the lesion surrounding the airway wall before and after treatment. The complications were also recorded during follow-up. Results: Eight patients underwent successful placement of a total of 11 radioactive stents (2 straight-type stents, 2 L-shape stents, and 7 Y-shape stents, all loaded a total of 243 radioactive particles). Displacement of stents took place within 2 weeks in 2 patients, who were managed with re-stenting and fixation. No further displacement occurred during follow-up. The median time to stent removal was 2.9(interquartile range: 2.3,3.0) months. After stent placement, the dyspnea index was significantly decreased compared with pre-treatment level (mean: 0.1 vs. 3.4, t= 8.881, P< 0.001). Bronchoscopic re-assessment showed that the residual tumor within the airway was detected in only one patient and that the tumor completely disappeared in the remaining 7 patients. Treatment with stents loaded with radioactive particles yielded smooth and pale airway mucosa with formation of partial scar formation. Chest computed tomography re-assessment demonstrated significantly larger luminal diameter than that before treatment (mean: 13.1 mm vs. 3.3 mm, t= -7.839, P< 0.001). The airway wall thickness was notably reduced after treatment (mean: 4.3 mm vs. 14.4 mm, t= 7.620, P< 0.001). The lesions surrounding the airway wall completely disappeared in 7 patients and decreased for more than 50% in a single patient. The median duration of follow-up was 28.0(interquartile range: 24.8,31.5) months. Recurrence of tumor was documented in a single case within 2 years. Six patients did not experience recurrence within the 2-year follow-up period. No death or severe complications were recorded during follow-up. Conclusion: The (125)I radioactive stent is effective for dilating the stenotic airway and ameliorating the symptoms, and thus might be an effective and safe method for the treatment of TACC. Further studies that explore the efficacy of stents loaded with (125)I particles are needed.

Aim: Adenoid cystic carcinoma is a rare tumor of head and neck region and its development in the thoracic region is even less frequent. This implies the absence of guidelines for therapeutic management and a consequent case-by-case approach. The role of radiotherapy is not yet clearly defined, but intensity-modulated radiotherapy allows for improved organ-at-risk sparing. Materials & methods: We have collected the cases of four patients treated at our institutions by the means of intensity-modulated radiotherapy, after endoscopic resection. Results & conclusion: Patients treated achieved long-term disease control of about 5 years, with a minimal acute toxicity. Longer follow-up is needed to drain conclusion on the impact of this treatment on overall survival.

Objective: Synthetic tracheal grafts seeded with autologous bone marrow-mononuclear cells (BM-MNCs) have been described as becoming living and functional grafts representing a promising option for tracheal replacement for pathologies unamenable by segmental resection or autologous repair. This study aimed to present the first long-term follow-up of these procedures in humans., Methods: We retrospectively analyzed 3 patients who received synthetic tracheal grafts seeded with BM-MNCs implanted., Results: Patient 1 was a 37-year-old man with mucoepidermoid carcinoma, the first-ever human to receive a synthetic tracheal graft seeded with BM-MNCs. Patient 2 was a 30-year-old man with adenoid cystic carcinoma, and patient 3 was a 22-year-old woman with an iatrogenic tracheal injury. All patients developed graft-related complications necessitating multiple surgical reinterventions. Patient 1 was hospitalized for 8 months before dying from respiratory failure secondary to graft dehiscence 32 months after implantation. Patient 2 died 3.5 months after implantation from undisclosed causes. Patient 3 received a second synthetic tracheal graft after 11 months and an allogeneic trachea and lung transplantation 45 months after the primary implantation. Patient 3 underwent 191 surgical interventions after the primary implantation and spent 55 months in the intensive care unit before dying from airway bleeding. All patients' bronchoscopic, histologic, and radiologic investigations demonstrated graft-associated complications, including anastomotic fistulae and obstructive granulation tissue, without graft vascularization, mucosal lining, or integration into adjacent tissues., Conclusions: Synthetic tracheal grafts seeded with BM-MNCs do not become living functional tracheal grafts and lead to debilitating complications and death., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

BACKGROUND Adenoid cystic carcinoma of trachea (ACCT) is an unusual low-grade tumor from the tracheal and bronchial wall glands. The histological structure and biological behavior of ACCT are similar to that of tumors found in the salivary glands. ACCT occurs most commonly in the upper trachea, being found in the articular cartilage in the posterior aspect of the trachea. CASE REPORT We describe the case of a 62-year-old male ex-smoker 25 years/pack (abstinence 20 years ago), who began with mild dyspnea 2 years ago, with intense evolution and cough. He was evaluated as an outpatient and was treated for sinusitis and later bronchitis after pulmonary function tests. With the worsening of the aforementioned symptoms, he sought prompt care, with hospitalization and computed tomography (CT) of the chest being indicated, showing an expansive lesion in the anterior wall of the trachea with an extension of approximately 3.1 cm, ending at the level of the carina, measuring 3.4×2.8 cm, with moderate stenosis of the tracheal lumen, with an exophytic component to the mediastinum. Bronchoscopy of the lesion was done, and later immunohistochemistry showed a probable pleomorphic adenoma. However, in a new analysis, after the removal of the surgical specimen, it was observed that it was a ACCT. CONCLUSIONS ACCT is a rare tumor that should be diagnosed as soon as possible in order to ensure its best prognosis. Moreover, it is evident that the analysis of the surgical specimen is sovereign to immunohistochemistry with regard to histological typing.

Background: Only a small series of studies has investigated primary tracheobronchial adenoid cystic carcinoma (TACC), and its prognosis and prognostic factors have not been well defined., Methods: Patients with TACC diagnosed histologically between January 1967 and December 2017 at our institution were retrospectively analyzed., Results: A total of 191 consecutive patients were included in our study. One hundred sixty-three patients underwent surgery, while the remaining 28 patients received nonoperative therapy. The 5-year, 10-year, and 20-year survival rates of the surgical group were 85.00%, 63.40%, and 47.00%, while the 5-year and 10-year survival rates of the nonoperative group were 63.70% and 46.40%, respectively. The multivariate analysis showed that the complaint duration (<7 months or ≥7 months) and treatment methods (R0 resection, R1 resection with radiotherapy, R1 resection without radiotherapy) were independent prognostic factors for disease-free survival of the R0/1 (resection with no residual tumor or microscopic residual tumor) resected patients, while the tumor size (≤3 cm or >3 cm) and treatment methods were independent prognostic factors for the overall survival. Sixty-four (45.1%) patients in the R0/1 group experienced recurrence/progression, and compared with chemotherapy or supportive treatment, local treatment significantly improved the prognosis of these patients (P < .050)., Conclusions: The complaint duration and tumor size are independent prognostic factors for disease-free survival and overall survival in TACC, respectively. Complete resection of the primary tumor, postoperative adjuvant radiotherapy for patients with positive margins, and local treatment after postoperative recurrence or progression may contribute to better survival., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Adenoid cystic carcinoma (ACC) of the trachea and bronchus is rare, representing 1% of all respiratory tract cancers. We presented our experiences in treating tracheal-bronchial ACC and the results of long-term surveillance., Methods: We conducted a retrospective study of treating tracheo-bronchial ACC. From 2009 to 2014, 42 patients presented to our department. All of them received surgical resection and adjunctive therapy., Results: R0 resections were achieved in 33 patients, whereas 15 patients whose lesions spread outside the tracheo-bronchial lumens. Nine patients had R1 resections followed by radiation and chemotherapy. The 5 year survival rate of R1 resection group showed no difference compared to the R0 resection group, but the 5 year disease-free survival rate showed difference in extra-lumenal invasion (ELI) and non-ELI group (P = 0.0357 < 0.05), although no difference was seen in the overall survival rate in these two groups., Conclusions: ACC of the trachea and bronchus is a rare and low-to-moderate grade malignant tumor. When the R0 resection is over risky or may cause mortal complication, the R1 resection with adjunctive therapy is acceptable for patients to obtain a promising prognosis, whereas pathological ELI is an adverse prognostic indicator., (© 2019 John Wiley & Sons Australia, Ltd.)

Background: Primary adenoid cystic carcinomas (ACCs) of the trachea are rare tumors of the central bronchial system. In patients presenting with unresectable tumors, severe comorbidities, or incomplete surgical resection, definitive radiotherapy is currently the recommended treatment. Irradiation with carbon ions (C 12 ) has shown promising local control (LC) and survival rates in cases of ACCs of the head and neck. No data on the therapeutic efficacy of C12 radiotherapy in treating tracheal ACC has been published., Methods: All patients with histologically confirmed ACC of the trachea treated with surgery and/or radiation treatment at Heidelberg University Hospital between 1991 and 2017 were included in this analysis. Patient and treatment characteristics, short- and long-term toxicity after radiotherapy, overall survival (OS), freedom from local progression (FFLP), and freedom from distant progression (FFDP) were prospectively acquired and retrospectively analyzed., Results: Thirty-eight patients (23 women and 15 men) with a median age of 51 were treated by surgery (n = 20) and/or radiotherapy with either C 12 (n = 7) or photons (n = 24). Of these patients, 61% presented with locally advanced (stage 4) ACC. The median follow-up for all patients was 74.5 months. The 5-year OS for all patients was 95% (10-year: 81%). The 5-year FFLP and FFDP were 96% (10-year: 83%) and 69% (10-year: 53%), respectively. In patients who underwent surgery alone, the 5-year OS was 100% (10-year: 80%). The 5-year FFLP and FFDP were 100% (10-year: 100%) and 80% (10-year: 60%), respectively. In patients who underwent radiotherapy alone, the 5-year OS was 100% (10-year: 83%). The 5-year FFLP and FFDP were 88% (10-year: 44%) and 67% (10-year: 34%), respectively. In patients who received multi-modal treatment including surgery and adjuvant radiotherapy, the 5-year OS was 84% (10-year: 84%). The 5-year FFLP was 100% (10-year: 100%) and the 5-year FFDP was 65% (10-year, 65%)., Conclusions: The long-term prognosis is favorable if surgery is performed. In cases of an incomplete resection, good OS can still be achieved following adjuvant radiotherapy. For radiotherapy, irradiation with C 12 shows promising first results. However, more data is needed to prove the long-term advantage of C 12 over photons., Trial Registration: The ethics committee of the Heidelberg University Hospital approved the retrospective data analysis (S-174/2019).

While adenoid cystic carcinoma is a common malignancy to arise within the salivary glands (21.9%) (Kokemueller et al. in Int J Oral Maxillofac Surg 33:25-31, 2004), it is seldom encountered as a tracheal mass and represents < 1% of all lung cancers. Tracheal tumors are an uncommon cause of dyspnoea due to their relatively rare occurrence (Baydur and Gottlieb in JAMA 234:829-831, 1975). They pose considerable diagnostic challenge clinically as their symptoms mimics more commoner ailments like asthma and chronic bronchitis. As they carry good prognosis, a timely diagnosis is highly warranted for appropriate therapeutic intervention. We describe a rare autopsy case of an adenoid cystic carcinoma in a middle-aged gentleman who presented with severe breathlessness and dyspnoea and succumbed to his illness after a brief hospital stay. Because the symptoms were non-specific, he was treated on lines of asthma and bronchitis.

Introduction: Primary tumours of the trachea are very rare and may develop from the tracheal salivary glands., Case Reports: We describe four patients operated on in our service between 2010 and 2017 of whom two had an adenocystic carcinoma, one a mucoepidermoid carcinoma and one a pleomorphic adenoma of the trachea. All were treated by resection and tracheal anastomosis with clear margins in three cases. The malignant cases received adjuvant treatment consisting of radiotherapy in one case and radiochemotherapy in the second. Immediate postoperative recovery was uncomplicated in all patients. One death followed the developement of post irradiation tracheal stenosis two years after surgery in a patient with an adenocystic carcinoma where the resection margins were invaded by tumour., Conclusions: Resection and anastomosis of the trachea remains the best therapeutic option with a better prognosis when the resection is complete., (Copyright © 2019 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Background: Adenoid cystic carcinoma of the trachea is a rare tumor, characterized by slow growth and low rate of local and distant metastasis. When achievable, complete surgical resection represents the optimal treatment approach, with the highest results in terms of overall survival. Radiation therapy is a reasonable alternative in cases of inoperable disease., Case Presentation: We report a case of an 82-year-old white man affected by primary adenoid cystic carcinoma of the trachea, treated with debulking surgery and radiotherapy on the residual disease. A three-dimensional conformal radiation therapy was conducted. The total dose amounted to 70 Gy, administered in 35 fractions of 2 Gy. The medium doses given to the esophagus and lungs were 23 Gy and 4.2 Gy respectively. The maximum dose delivered to the spinal cord was 31 Gy with satisfactory results in terms of local control of the disease., Conclusion: A combined approach of surgical resection followed by radiotherapy on the residual disease provided an excellent result in terms of disease control, quality of life, and overall survival in a patient with locally advanced tracheal adenoid cystic carcinoma.

Adenoid cystic carcinoma or cylindroma is a rare tumor of the trachea which arises from mucous secreting cells. Its evolution and growth are slow but it is often fatal in the absence of radical treatment. The latter relies on a surgical sleeve resection of the pathological area, with a tracheo-tracheal anastomosis. Therapeutic bronchoscopy plays a crucial role in the clinical improvement of patients before surgery which will be performed under better conditions. For inoperable patients, radiotherapy has proved its efficacy, unlike conventional chemotherapy. The finding of biomarkers expressed by tumor cells could lead to target therapies. This case report illustrates the efficient combination of therapeutic bronchoscopy and surgery for the treatment of adenoid cystic carcinoma affecting central airways., (Copyright © 2018 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Primary tumours of the trachea are rare. Adenoid cystic carcinoma (ACC) constitutes less than 1% of these tumours. They occasionally masquerade as asthma. We are presenting a case of a young female, who had been treated for 3 years as asthma. She presented in emergency with severe respiratory difficulty, stridor and decreasing saturation of 85% on room air. Two weeks back, she had a CT scan in a local hospital, but they did not notify the tracheal mass. Her clinical picture and CT scan helped us diagnose the case. She was treated in emergency with primary resection of tracheal mass and anastomosis. The tumor was diagnosed as ACC. Postoperative recovery was uneventful. Continuity of the trachea was restored and adjuvant radiotherapy was given.

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.

Rationale: Primary tracheal adenoid cystic carcinoma of the trachea primary is a rare neoplasm and commonly misdiagnosed. Lung isolation during surgery and ventilation pose a tremendous challenge to anesthesiologists., Patient Concerns: The authors describe a novel technique of lung isolation and ventilation with a Univent tube during thoracoscopic mediastinal tracheal resection and reconstruction in a female patient., Diagnoses: Primary tracheal adenoid cystic carcinoma, nonsmall cell carcinoma., Interventions: In this case, tracheal resection and reconstruction were performed. A bronchial blocker of the Univent tube was used as a guide to manipulate the depth of endotracheal tube., Outcomes: The intermittent 1-lung ventilation was established successfully. The patient recovered uneventfully and discharged after 10 days., Lessons: The advantages of approach include a stable airway management without occupying the contracted space of thoracoscope and no potential risk of trapping or barotraumas., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Aims: Adenoid cystic carcinoma (AdCC) is a malignant epithelial neoplasm that occurs rarely in the lower respiratory tract (LRT). AdCC at various sites is associated with the novel fusion transcript MYB-NFIB, along with the overexpression of the Myb protein. The expression of the Myb protein in AdCC of the LRT has not been evaluated much., Study Design: Cases of AdCC of the LRT diagnosed on cytology or histology were retrieved from our institutional archives. c-Myb expression was analyzed on immunocytochemistry/immunohistochemistry (ICC/IHC) and was correlated with clinicopathological parameters., Results: Twenty-three samples of AdCC originating from the LRT were included in the study. Four cases were diagnosed on cytology, 3 of which had corresponding histology specimens. The remaining 19 cases had either biopsy or resection. Most of the patients presented with endobronchial mass. The mean age was 49.4 years and a male predominance was seen. ICC and IHC for c-Myb showed positivity in 75 and 59% of the cases, respectively. Western blot was used to validate IHC results., Conclusion: AdCC of the LRT is rare and hence poses diagnostic difficulty. Cytology smears can be utilized for c-Myb ICC. The presence of c-Myb immunopositivity in most cases may possibly make Myb a diagnostic biomarker and a therapeutic target for personalized treatment., (© 2016 S. Karger AG, Basel.)

We report the case of a 63-year-old woman with adenoid cystic carcinoma of the lower trachea treated by resection of 11 of the 18 cartilaginous rings (61%) of the total length. The little remaining membranous portion of the carina was sewn up to create a margin for anastomosis. The anastomotic sites could be approximated by the mobilization of the cervical trachea and the left main bronchus, pulling across the traction sutures, and anteflexion of the neck. The patient's postoperative course was uneventful without any complications associated with anastomosis. Because both the proximal and distal margins showed microscopic tumors, radiation therapy was performed with 50 Gy 2 months after surgery. The patient has a good social life without recurrence 20 months after surgery.

Primary malignant tracheal tumors are not common and adenoid cystic carcinoma (ACC) of trachea is very rare. The diagnosis is often delayed due to the atypical symptoms. We report an extremely rare case of ACC of proximal trachea, in a 55-year-old female who presented with a 12 month history of progressive dyspnea. Laryngoscopy and computed tomography revealed a broad-based polypoidal mass arising from posterior wall of the proximal trachea. Biopsy confirmed the diagnosis of ACC. The patient underwent a complete surgical resection and post operative radiotherapy. Six months follow-up of the patient did not reveal local recurrence or distant metastases. The literature of tracheal ACC is reviewed.

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.)

Aims: To review retrospectively 21 cases adenoid cystic carcinoma of the tracheobronchial tree (TACC) with emphasis on their clinical and pathologic features, treatment and the possible prognostic factors., Methods and Results: 21 cases TACC diagnosed by surgical biopsy or resection at the Peking Union Medical College Hospital (PUMCH) over 10 years. Patients aged 24-69 years (median, 49 years), 6 men/15 women. Cough (18/21), dyspnea (14/21) and hemoptysis (10/21) were the most frequent manifestations. 15 patients had tumors in trachea. Ten patients had pathologically positive margin (n = 11). Immunohistochemically, BCL-2, CD117, P16, type IV collagen, SMA and P63 were positive (20/20); GFAP was focally positive (4/20); TTF-1 and P53 were negative (0/20). Ki-67 index ranged from 2% to 35%. Fifteen patients had followed up, 13 of which received postoperative radiotherapy. The median relapse-free survival (RFS) was 56.9 months and the 5-year RFS was 48.6%. By univariate analysis, postoperative radiotherapy had favorable prognostic significance (P < 0.05)., Conclusions: TACC, which is mainly located in primary trachea or bronchus, is difficult to be detected at early stage. The tumors are not likely to be completely removed by surgery, and postoperative radiotherapy is helpful for reducing the likelihood of recurrence and metastasis.

Adenoid cystic carcinoma (ACC) of the trachea is rare, it represents 1% of all respiratory tract cancers. It's generally considered as a slow-growing, with pronlonged clinical course. Most patients present with dyspnea, and the symptoms often mimic those of asthma or chronic bronchitis Surgical resection is the mainstay of treatment often combined to radiotherapy because of close surgical margins. When surgery isn't possible, most tumors respond to radiotherapy alone wich often results in long periods of remission We report two cases of primary ACC of trachea: a 49 year old male presented a distal unresectable tracheal ACC treated with chemo-radiotherapy who developed a recurrence and died 7 years after the diagnosis. And a 50 years old female with a proximal tracheal tumor treated by surgical resection and end- to- end anastomosis followed by adjuvant radiotherapy. At 10 months follow-up, our patient shows no evidence of disease with negative histological findings.

Malignant involvement of the trachea predominantly results from direct spread of neighboring tumors to the tracheal wall. Primary tracheal malignancies show a low incidence of approximately 0.1 in every 100,000 persons per year, squamous cell carcinomas and adenoid cystic carcinomas accounting for about two-thirds of adult primary tracheal tumors. The etiology of squamous cell carcinoma and its premalignant lesions is strongly associated with tobacco smoking. Patients with tracheal malignancies show an unfavorable prognosis, with reported 5- and 10-year survival rates of 5% to 15% and 6% to 7%, respectively, for all types of tracheal carcinoma., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Primary tracheal tumors are uncommon, making up only 0.2% of all respiratory malignancies. Adenoid cystic carcinoma (ACC) is the most common tumor, accounting for about 30% of primary tracheal tumors. It is often difficult to manage these tumors surgically, due to its expansion and submucosal invasion, and furthermore, due to the patient's condition. Thus, it is essential to perform palliative treatment in order to maintain the airway through and to control the progress. Herein, we report a case of ACC treated by repeated bronchoscopic argon plasma coagulation (APC) as palliative therapy.A 71-year-old Japanese male was referred to our hospital's emergency department for dyspnea. Bronchoscopic examination revealed expanded intraluminal obstructive bronchial tumors from the left bronchus to right. The patient had undergone bronchoscopic APC treatment several times. The obstruction was improved, and no worsening was seen for 26 months. Repeated bronchoscopic APC as a palliative therapy is promising and useful therapy without heavy adverse reactions to control the tumor growth.

Dedifferentiated adenoid cystic carcinoma is an extremely rare and highly aggressive tumor. We describe the first reported case of dedifferentiated adenoid cystic carcinoma of the trachea and analyze the expression profiles of mammalian target of rapamycin pathway proteins. A 66-year-old Japanese man was incidentally found to have stenosis of the trachea, and a bronchial biopsy revealed low-grade adenoid cystic carcinoma. The resected specimen revealed dedifferentiated adenoid cystic carcinoma, which was composed of conventional low-grade adenoid cystic carcinoma with tubular and cribriform patterns, and a dedifferentiated carcinoma component (poorly differentiated adenocarcinoma). Immunohistochemical study showed that mammalian target of rapamycin and 4E-BP1 were expressed in both components; however, phosphorylated 4E-BP1 was expressed only in the dedifferentiated carcinoma component. This report clearly demonstrates that mammalian target of rapamycin pathway proteins were activated in dedifferentiated carcinoma. Mammalian target of rapamycin is a central protein involved in carcinogenesis, and administration of its inhibitors prolonged survival in some types of carcinoma. Therefore, mammalian target of rapamycin inhibitors may be a potential candidate for treatment of this highly aggressive carcinoma., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Circumferential airway resection with primary anastomosis has been widely adopted as a treatment for adenoid cystic carcinoma (ACC) of the trachea. However, carinal resection is a complicated procedure with high mortality and morbidity rates. We describe a technique of non-circumferential tracheal resection performed to treat ACC arising from the lower membranous trachea adjacent to the carina. The tumor was resected while preserving the tracheo-carinal cartilage. A silicone Y-stent was placed at the bifurcation to ensure airway patency before closing the defect. The airway defect, measuring 4 × 2.5 cm, was closed using an autologous pericardial patch and pedicled latissimus dorsi muscle flap. These procedures were technically easy, and no postoperative airway complication occurred.

Background: Adenoid cystic carcinoma of the respiratory tract is a rare form of malignant neoplasm that arises from salivary glands. A surgical resection with or without radiotherapy is commonly recommended. For inoperable patients, radiotherapy and endobronchial treatment, which include laser and stent insertion, are important therapeutic modalities., Aim: To summarize our experience in adenocystic carcinoma of the respiratory tract., Methods: Seven patients with adenoid cystic carcinoma were diagnosed and treated in the Pulmonary Institute at Rabin Medical Center, a tertiary care hospital in central Israel in 2000-2006., Results: Three patients were men and four were women, with a presenting age at diagnosis ranging from 31 to 79 years. All the patients were nonsmokers. Major symptoms at presentation included dry cough, dyspnea and hemoptysis. Only two patients were operable at time of presentation and 4 patients had local and distant metastases. In 6 patients the tumor was presented in the trachea with marked obstruction of the tracheal lumen. In 4 patients coexisting lesions in the main bronchus were also found. Two patients with non-metastatic disease underwent surgical resection with external radiation. Multiple laser therapy and brachytherapy were performed in 4 patients. Two patients with severe airway stenosis that was resistant to radiotherapy were treated with laser and intratracheal stent replacement., Conclusions: Endobronchial therapy that includes brachytherapy, laser and stent insertion, is a useful therapeutic modality for the treatment of patients with adenoid cystic carcinoma of the respiratory tract who are resistant to radiotherapy.

Adenoid cystic carcinoma (ACC), commonly from salivary glands, is known for its insidious local growth and usually protracted clinical course. ACC developing from non-salivary glands (i.e., non-salivary ACC) is heterogeneous, and its clinicopathological features remain poorly defined. Patients treated for ACC in a single institution between 1995 and 2007 were included in this study. Immunohistochemical evaluation of Ki-67, E-cadherin, p16, and cyclinD1 was performed. The prognostic significance of clinical and immunophenotypic markers was evaluated. 83 cases of salivary ACC and 24 cases of non-salivary ACC were included. The expression levels of Ki-67 (54.8%), E-cadherin (90.4%), p16 (32.9%), and cyclinD1 (19.2%) between ACCs present at various sites were not different. Sinonasal, lacrimal, and tracheobronchial ACCs had significantly worse outcomes than those of ACC of the major salivary glands. Postoperative radiotherapy reduced the recurrence rate of patients with a negative resection margin (P=0.028). Older age (age >60 years), advanced stage, positive resection margin, high histological grade, and high expression of Ki-67 were significantly correlated with poor prognosis. In conclusion, the site of origin plays a role in the prognosis of ACC, in which positive resection margin and advanced stage are possible factors underlying the differences in outcomes., (Copyright © 2011 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Introduction: Primary carcinomas of the trachea are rare tumors, occurring at a rate of 2.6 new cases per 1,000,000 people per year. This study investigates the large observational cohort of patients recorded in the NCI Surveillance, Epidemiology, and End Results (SEER) 1973-2004 database, and provides information regarding epidemiology, treatment, and prognosis., Methods: The SEER database was investigated, and all patients for whom primary tracheal carcinoma was the first and only cancer were investigated. Demographic information was investigated. The cohort was analyzed for variables effecting survival, including age, gender, race, histology, extent of disease, extent of surgery, use of radiation, and year of diagnosis., Results: Between 1973 and 2004, 578 cases of primary tracheal carcinomas were reported in the SEER database. There were 322 men (55.7%) and 256 women (44.3%). Squamous cell carcinoma was the predominant histology, representing 259 tumors (44.8%). Adenoid cystic carcinoma (ACC) was the second most common tumor (16.3%). Localized, regional and distant disease was found in 140 (24.2%), 212 (36.7%), and 108 (18.7%), respectively. Twenty percent of the patients did not undergo staging. Patients with localized disease had a better prognosis than those with regional (P = 0.001) or distant disease (P = <0.001).A significant fraction of patients did not receive cancer directed local therapy; 34.3% did not undergo surgery and 29.1% did not receive any kind of radiation therapy. There was a statistically significant improved survival for patients who underwent any type of surgery in comparison with patients who did not undergo cancer directed surgery. There was no statistical benefit for patients who underwent radiation therapy.General overall 5-year survival for all patients was 27.1% (95% CI: 23.1-33.3%). Patients with localized disease had a better outcome than patients with regional or distant disease with an overall 5-year survival of 46% (95% CI: 37.3%-55.8%). Squamous cell carcinoma tumors had worse outcomes than any other histologic type, with a 5 year overall survival of 12.6% (95% CI: 8.4-17.6%). In contrast, 5-year overall survival for AACs was relatively good at 74.3% (95% CI: 63.1-82.5). For localized disease, 5-year survival was 24.7% (95% CI: 12.8-38.7%) for squamous cell carcinoma versus 90.5% (95% CI: 73.3-96.8%) for ACCs (P < 0.001)., Conclusion: Primary tracheal tumors are very uncommon; squamous cell carcinoma is the most common histologic type, followed by ACCs. General 5-year overall survival is poor, though localized disease has better survival when compared with regional or distant disease. There is a remarkable difference in survival between squamous cell carcinoma and ACC.

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.