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Abstract: Schwannoma is a benign tumor originating from Schwann cells. It commonly occurs in the head, neck, and extremities, but rarely occurs in the trachea. Tracheal schwannoma is usually asymptomatic. We reported the 18 F-FDG PET/CT findings of a 61-year-old man with bronchoscopically biopsy-proven schwannoma, which presented challenges in differentiation from certain benign tumors and low-grade malignancies in the trachea., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Primary tracheal carcinoma is an exceptionally rare and life-threatening disease that presents significant diagnostic and therapeutic challenges. Delayed diagnosis due to misinterpretation of airway obstruction symptoms often leads to poorer prognoses for patients. This study aimed to explore the clinical manifestations and multidisciplinary treatment approaches for primary tracheal carcinoma in Bangladesh, with a focus on recent advancements in diagnosis and treatment., Methods: A retrospective observational study was conducted at Bangladesh Specialized Hospital Limited, involving patients aged over 30 who were diagnosed with tracheal carcinoma and underwent multidisiplinary treatment from July 2018 to June 2019. Data were collected through patient interviews and medical record reviews. Descriptive and inferential statistical analyses were performed to examine demographic characteristics, histological variations, tumor locations, clinical signs and symptoms, treatment approaches, and outcomes., Results: The study illuminated varied clinical presentations and the successful application of multidisciplinary approaches among the 13 patients. Invasive squamous cell carcinoma and adenoid cystic carcinoma were the predominant histological subtypes. Symptomatology, including dyspnea, cough, and hemoptysis, highlighted the challenge of early detection. Despite the rarity and intricacies associated with primary tracheal carcinoma, the multidisciplinary strategy yielded generally positive outcomes, as evidenced by a 1-year survival rate of 92.31% and a 5-year survival rate of 76.92%. Kaplan-Meier survival curves underscored the superior efficacy of surgical interventions over non-surgical approaches., Conclusion: Despite some limitations, this study contributes crucial insights into the nuanced management of primary tracheal carcinoma in the Bangladeshi context. The demonstrated success of the multidisciplinary strategy, especially surgical interventions, accentuates the importance of definitive resection. The lone case of local recurrence emphasizes the necessity for vigilant follow-up., (© 2024 The Author(s). Cancer Reports published by Wiley Periodicals LLC.)

Introduction: The surgical management of patients diagnosed with papillary thyroid carcinoma (PTC) and tracheal invasion has been a subject of ongoing discussion, particularly regarding the approach to tracheal functional reconstruction. The objective of this study was to examine the surgical technique and prognosis of PTC patients with tracheal invasion., Materials and Methods: This study employed both univariate and multivariate Cox proportional hazard models to determine predictive factors that affect the progression-free survival (PFS) of PTC patients with tracheal invasion. Cox regression analysis was conducted by using R software version 4.3.1., Results: In our study, we included 247 patients with T4a PTC. Among them, 146 patients (59.1 %) were classified as Shin I, 57 patients (23.1 %) as Shin II-III, and 44 patients (17.8 %) as Shin IV. Patients in the Shin I group underwent shaving of the tumours in the airway. The preferred surgical methods in the Shin II, III and IV groups were window resection (66.7 %), sleeve resection (34.8 %) and partial tracheal resection and skin fistula (61.8 %), respectively. Multivariate analysis demonstrated that neither tracheal surgery nor reconstruction procedures had an impact on PFS in T4a PTC patients with tracheal invasion. The 5-year DSS rate for patients receiving radioiodine (RAI) therapy was 87.3 % (p = 0.033)., Conclusion: This study confirmed that tracheal surgery and reconstruction methods had no impact on PFS in T4a PTC patients with tracheal invasion in different Shin groups. Furthermore, RAI therapy has the potential to increase the survival rate of patients with preoperative distant metastasis of T4a PTC., Competing Interests: Declaration of competing interest There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported., (© 2024 Published by Elsevier Ltd.)

Objective: Primary tracheal tumors are very rare and their management is not definitely established. Due to its rarity, providing patient care in terms of optimal management poses a considerable challenge. The purpose of this study was to investigate treatment outcomes in patients with these rare tumors., Methods: We carried out a retrospective analysis of 89 patients with primary tracheal tumors treated at the Maria Sklodowska-Curie National Research Institute of Oncology in Warsaw, Poland, over sixteen years. The study assessed patient demographics, tumor characteristics and treatment. Different treatment options were compared in terms of overall survival, disease-free survival, and progression-free survival., Results: A total of 89 patients were included in the study. In the group presented, 45 patients underwent primary radical treatment and 44 were qualified for palliative treatment. Surgical resection was performed in 13 patients out of radically treated patients. The 5 year OS rates in the group of patients who underwent radical treatment and in the group of patients who underwent palliative treatment were 45.9% and 2.3%, respectively. In the group of patients who underwent radical surgical treatment, the 5 year OS was 76.9% compared to 35.8% in the group of patients who underwent nonsurgical treatment., Conclusion: A multidisciplinary team should decide treatment options, including in-depth consideration of surgical treatment options., (© 2024. The Author(s).)

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.)

Introduction: Primary tracheal acinic cell carcinoma (ACC) is an exceptionally rare malignancy, posing challenges in understanding its clinical behavior and optimal management. Surgical resection has traditionally been the primary treatment modality, but we present a compelling case of tracheal ACC managed with endotracheal intervention, challenging conventional approaches., Patient Concerns: A 53-year-old woman presented with shortness of breath, cough, and hemoptysis. Enhanced computed tomography revealed an obstructive tracheal lesion, leading to her referral for further assessment., Diagnosis: Microscopic evaluation, immunohistochemistry, and clinical assessments confirmed primary tracheal ACC, an exceedingly rare condition with limited clinical insights., Interventions: We utilized rigid bronchoscopy to perform endotracheal intervention, successfully resecting the tumor and restoring tracheal patency. Postoperatively, the patient received no radiotherapy or chemotherapy., Outcomes: The patient achieved complete recovery, with 24-month follow-up examinations indicating no recurrence or metastatic disease. Only minimal scar tissue remained at the resection site., Conclusion: This case demonstrates the potential of endotracheal intervention as a curative approach for primary tracheal ACC, minimizing invasiveness and preserving tracheal function. Collaborative research efforts and extensive case reporting are crucial for advancing our understanding of this rare malignancy and optimizing treatment strategies for improved patient outcomes., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

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 Primary tracheal schwannoma is a rare neurogenic tumor. Early stage presents with nonspecific symptoms, and asthma is sometimes misdiagnosed. However, as the tumor grows, it presents with obstructive symptoms of the tracheal lumen. This tumor has been managed by open resection surgery until recently, when endoscopic excision became an option. The endoscopic excision reduces complications, operative time, and postoperative recovery period and is indicated in nonrecurrent surgical cases in which tumors are up to 2 cm in size, are pedunculated, and have no extratracheal extension, or in cases of poor cardiopulmonary status. We present a rare case of primary tracheal schwannoma managed by endoscopic excision. CASE REPORT A 37-year-old man was referred to our clinic with progressive shortness of breath and wheezing that started 3 months prior to presentation. Computed tomography showed a well-defined rounded, solid intraluminal tracheal mass at the proximal segment (at the level of the thoracic inlet). There was no extratracheal extension or enlarged cervical lymph nodes. The patient underwent endoscopic excision of the mass. A sickle knife, micro scissor, and suction diathermy were used for incision, stripping, and hemostasis done through the tumor pedicle. The first postoperative visit after 2 weeks showed subjective symptom improvement, and the flexible bronchoscope showed a completely healed surgical site with patent airway. Histopathological examination and immunohistochemistry confirmed the diagnosis of primary tracheal schwannoma. CONCLUSIONS Primary tracheal schwannoma is rare. An endoscopic excision is an excellent option, but patients need to be appropriately selected and followed up to avoid recurrence.

The diagnosis and management of tracheobronchial papilloma is challenging due to its rarity, and non-specific presenting symptoms. Small percentage undergoes malignant transformation. Herein, we report an unusual case of tracheal papilloma initially misdiagnosed as chronic obstructive pulmonary disease (COPD) in 36-year-old male with triple Y syndrome. It was successfully treated with local debridement and brachytherapy. To the best of our knowledge, this is the first description of brachytherapy for such a condition., (© 2023. The Author(s).)

Central airway obstruction due to tracheal tumors presents unique challenges to the anesthesiologist. We present the case of a 44-year-old male taken to the OR for biopsy and resection of an undiagnosed tracheal mass. Intraoperative management was complicated by bleeding and significant hemodynamic instability, necessitating rapid surgical and anesthetic intervention. This ultimately led to abortion of surgical resection. Pathologic examination revealed a primary tracheal plasmacytoma, a rare type of tracheal tumor. Here, we describe anesthetic and hemodynamic considerations for a tracheal plasmacytoma. We discuss the approach to airway management in variable intrathoracic tracheal obstruction and the unpredictability of tracheal tumors.

Rationale: Salivary gland-type acinic cell carcinoma (ACC) is a low-grade malignancy. Primary ACC of the trachea and lungs is rare; here, we describe 1 such case. The histological morphology of tracheal ACC was similar to that of its salivary gland-associated equivalent. Because of its rarity, it is easily misdiagnosed as another type of tracheal or lung tumor. Microscopic analysis of pathological features and immunohistochemistry help diagnose primary ACC of the trachea and lungs., Patient Concerns: A 33-year-old female complained of shortness of breath and hemoptysis for 2 years, and reported the symptoms to have aggravated over the last 4 months. The patient was admitted to our hospital for further treatment. Enhanced computed tomography revealed a soft tissue density nodule shadow in the trachea, which was approximately 1.3 × 1.2 cm in size., Diagnoses: Based on the clinical information, morphological features, and immunohistochemistry, the pathological diagnosis was primary ACC of the trachea., Intervention: The tracheal lesion was resected with an electric snare, electrotomy, freezing, and an argon knife using a rigid bronchoscope., Outcomes: The patient's postoperative course was uneventful., Lessons: It is important to prevent misdiagnosis of this type of tumor as another type of lung tumor. Morphological and immunohistochemical features can be useful in diagnosing primary ACC of the trachea and lungs., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: Endobronchial masses such as mucoepidermoid carcinomas or carcinoid tumors are extremely rare in children and they usually originate from large bronchi. These lesions may cause wheezing and dyspnea with poor response to bronchodilators and mimic the airway obstruction caused by asthma., Case Study: We present the case of an 8-year-old girl with tracheal mucoepidermoid carcinoma who was treated as a difficult asthma case with high dose of inhaled corticosteroids., Results: The characteristic stridor, the lack of response to bronchodilators and to inhaled corticosteroid treatment, combined with the characteristic flow loop in spirometry and the hyperinflation seen on the chest radiograph, all raised the clinical suspicion of a tracheal lesion and indicated the need for flexible bronchoscopy. The bronchoscopy revealed a large lesion obstructing totally the trachea lumen. The latter finding was confirmed by chest high resolution CT. The mass was completely excised via sternotomy under cardiopulmonary bypass, and the pathologic examination showed a low-grade mucoepidermoid carcinoma of the trachea. One month after the surgery she was free of symptoms and her spirometry was normal., Conclusion: Tracheal lesions mimic the symptoms of airway obstruction caused by asthma and should be always be part of the differential diagnosis in young patients with no response to asthma treatment.

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.)

Abstract: Solitary non-Hodgkin lymphoma of the trachea is extremely rare. Herein, we reported a case of solitary laryngotracheal lymphoma in a 55-year-old man. He complained of cough, tachypnea, and dyspnea. 18F-FDG PET/CT showed a hypermetabolic lesion in the subglottic larynx and upper cervical trachea. The subsequent histology and immunohistochemistry of the laryngotracheal lesion tissue confirmed the diagnosis of non-Hodgkin lymphoma (mucosa-associated lymphatic tissue lymphoma)., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

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

Nuclear protein in testis (NUT) carcinoma is a rare and highly aggressive epithelial malignancy defined by rearrangement of the NUTM1 gene on chromosome 15q14. Histologically, NUT carcinoma is an undifferentiated carcinoma formed by sheets and nests of primitive and monotonous "round blue cells" with foci of abrupt keratinization in a subset. NUT carcinoma runs a fulminant clinical course and is almost always quickly lethal, with a median overall survival of only 6.7 months. There is no consensus regarding treatment for this disease, and most patients respond poorly to conventional chemotherapy and radiation. We report a case of NUT carcinoma in an African-American man who initially presented in 2009 with a tracheal mass at age 28. Although fluorescence in situ hybridization (FISH) assays for NUTM1 and BRD4 rearrangements were negative, he was diagnosed based on diffusely positive NUT immunostaining and BRD4-NUTM1 on RNA sequencing. Since his initial presentation, he has undergone multiple surgical procedures and radiation therapy. His tumor has recurred twice, but he has survived for 129 months and is currently alive without disease. Long-term survival of patients with NUT carcinoma is incredibly unusual, especially in patients with tumors that exhibit a BRD4 rearrangement. False negative FISH is a pitfall in diagnosing NUT carcinoma; NUT immunostaining and RNA sequencing are more sensitive diagnostic methods.

Objective: To investigate the potential value of 18 F-fluorodeoxyglucose (FDG) PET/CT in predicting the survival of patients with primary tracheal malignant tumors., Materials and Methods: An analysis of FDG PET/CT findings in 37 primary tracheal malignant tumor patients with a median follow-up period of 43.2 months (range, 10.8-143.2 months) was performed. Cox proportional hazards regression analyses were used to assess the associations between quantitative 18 F-FDG PET/CT parameters, other clinic-pathological factors, and overall survival (OS). A risk prognosis model was established according to the independent prognostic factors identified on multivariate analysis. A survival curve determined by the Kaplan-Meier method was used to assess whether the prognosis prediction model could effectively stratify patients with different risks factors., Results: The median survival time of the 37 patients with tracheal tumors was 38.0 months, with a 95% confidence interval of 10.8 to 65.2 months. The 3-year, 5-year and 10-year survival rate were 54.1%, 43.2%, and 16.2%, respectively. The metabolic tumor volume (MTV), total lesion glycolysis (TLG), maximum standardized uptake value, age, pathological type, extension categories, and lymph node stage were included in multivariate analyses. Multivariate analysis showed MTV ( p = 0.011), TLG ( p = 0.020), pathological type ( p = 0.037), and extension categories ( p = 0.038) were independent prognostic factors for OS. Additionally, assessment of the survival curve using the Kaplan-Meier method showed that our prognosis prediction model can effectively stratify patients with different risks factors ( p < 0.001)., Conclusion: This study shows that 18 F-FDG PET/CT can predict the survival of patients with primary tracheal malignant tumors. Patients with an MTV > 5.19, a TLG > 16.94 on PET/CT scans, squamous cell carcinoma, and non-E1 were more likely to have a reduced OS., Competing Interests: The authors have no potential conflicts of interest to disclose., (Copyright © 2021 The Korean Society of Radiology.)

Tracheal tumors are rare in children and manifest symptoms of airway obstruction. A 14-year-old boy with a 5-month history of dyspnea and wheezing was referred to our hospital. Although he had been initially diagnosed with bronchial asthma, computed tomography revealed tracheal tumors. Histologic examination showed only necrotic tissue. Thereafter, the systemic steroid treatment for bronchial asthma was tapered off. A second computed tomography scan revealed new lesions in the pancreas and lung. Biopsy of the pancreatic lesion revealed a diffuse large B-cell lymphoma. The patient was administered standard chemotherapy, following which he went into complete remission., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, 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.

A 38-year-old male patient presented to the ear, nose and throat department with shortness of breath over last 2 months. The CT scan of the neck and chest revealed a 3.3×3 cm tumour behind the right thyroid lobe extending into the tracheo-oesophageal (TO) groove with tracheal compression. The ultrasound scan of the neck and targeted fine needle aspiration followed by core biopsy raised a suspicion of Hodgkin's lymphoma. The patient underwent a right hemithyroidectomy and incisional biopsy of the right TO groove tumour. The histology confirmed a Hasenclever's three nodular sclerosing Hodgkin's lymphoma for which he received adjuvant chemotherapy. An incidental pT1a pN0 thyroid papillary microcarcinoma in the adjacent thyroid parenchyma was completely excised. This represents a case of TO Hodgkin's lymphoma, of which there are no current published case reports. We aim to raise awareness about this rare condition by sharing the diagnostic work up and successful management in a multidisciplinary team setting., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

We experienced a surgical case of a rare primary tracheal tumor. A 77-year-old woman visited a local clinic with chief complaints of coughing, wheezing, and discomfort in the throat. Computed tomography revealed a mass measuring approximately 1.5 cm in the mediastinal trachea, extending from the membranous portion of the trachea to the esophagus. Bronchofibroscopy showed a flat, smooth-surfaced, round mass arising from the membranous portion. Surgery was performed because of the possibility of airway obstruction and suffocation. Sleeve resection of five tracheal rings was performed via median sternotomy and interrupted suture was performed using 3-0 absorbable suture material. The postoperative course was favorable and there has been no evidence of recurrence. The pathological diagnosis was solitary fibrous tumor. A primary solitary fibrous tumor of the trachea is extremely rare. Here, we report this disease with a literature review.

Background: This report describes a case of solitary tracheal lymphoma in a 14-year-old alpaca mare., Case Presentation: The alpaca was referred for dyspnea and inspiratory noise. The clinical examination included complete blood cell count, blood chemistry, endoscopy, ultrasound, radiographs, and computed tomography (CT). A solitary tracheal intraluminal and juxtatracheal lymphoma was diagnosed by fine needle aspiration (FNA). The owner requested euthanasia due to the uncertain prognosis. At postmortem examination, the presence of solitary lymphoma without involvement of other organs was confirmed. Immunohistochemical analysis confirmed a B-cell origin., Conclusions: Although multicentric lymphoma is the most commonly described neoplasia affecting South American camelids (SAC), solitary forms of the disease may occur.

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.)

Background: Tracheal invasion is reported to occur in approximately one-third of the patients of locally advanced thyroid cancers. There is a paucity of data in literature with regard to the long-term outcomes of thyroid cancers with tracheal invasion., Methods: A total of 37 patients from our tertiary care center underwent radical surgery for tracheal involvement for differentiated thyroid cancers between the years 2002 and 2016. The variables pertaining to the demographics, clinical presentation, imaging, operative details and histopathology reports were captured from the patient's case records and analyzed., Results: Among the 37 patients, there were 21 males and 16 females. Majority of the patients (56.8%) were >55 years of age. Surgery (tracheal resection) was performed in the primary setting in 29 patients, whereas it was performed in a recurrent setting in 8 patients. As per the Shin classification, 3 patients belonged to Shin stage 1, 3 to Shin stage 2, 16 patients to Shin stage 3 and 15 patients to Shin stage 4. There was no 30 day postoperative mortality in our cohort. The median follow-up of our cohort was 175 months. The 5-, 10-, and the 15-year overall survivals of the entire cohort were 81.7%, 47.8%, and 35.9%, respectively., Conclusion: Our series shows favorable long-term oncological outcomes of selected patients of thyroid cancers with tracheal resection and adds to the limited long-term data available in literature., Competing Interests: None

For malignant tumours, treatment is rarely indicated in cases requiring mechanical ventilation management because such intensive care would engender a decrease in performance status. However, light sedation using dexmedetomidine might enable chemoradiation while accommodating activities of daily living. We experienced two cases of fatal tracheal invasion and airway stenosis of stage Ⅳ oesophageal cancer that were treated with chemoradiation or radiation under mechanical ventilation (one case was differential lung ventilation.) with dexmedetomidine alone and rehabilitation was performed under a ventilator. Early mobilisation by light sedation with dexmedetomidine can inhibit performance status decline attributable to mechanical ventilation. Bridging tracheal intubation with light sedation by dexmedetomidine for temporary chemoradiation therapy to reduce tumour volume might present a good alternative for patients with malignant tumour., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

Tracheobronchial masses encompass a broad spectrum of entities, ranging from benign and malignant neoplasms to infectious and inflammatory processes. This article reviews the cross-sectional findings of tracheal tumors and tumor-like entities, correlates imaging findings with histologic pathology, and discusses pearls and pitfalls in accurately diagnosing and classifying tracheal tumors and mimics., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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.)

Objective: Primary tracheobronchial tumors (PTTs) are rare heterogeneous lesions arising from any part of the tracheobronchial tree. Nonspecific symptoms may lead to delayed diagnosis that requires more aggressive surgical treatment. An analysis of cases collected by the International Network of Pediatric Airway Team was undertaken to ensure proper insight into the behavior and management of PTTs., Methods: Patients <18 years of age with a histological confirmation of PTT diagnosed from 2000 to 2015 were included in this multicenter international retrospective study. Medical records, treatment modalities, and outcomes were analyzed. The patient presentation, tumor management, and clinical course were compared between malignant and benign histotypes. Clinical and surgical variables that might influence event-free survival were considered., Results: Among the 78 children identified, PTTs were more likely to be malignant than benign; bronchial carcinoid tumor (n = 31; 40%) was the most common histological subtype, followed by inflammatory myofibroblastic tumor (n = 19; 25%) and mucoepidermoid carcinoma (n = 15; 19%). Regarding symptoms at presentation, wheezing (P = 0.001) and dyspnea (P = 0.03) were more often associated with benign growth, whereas hemoptysis was more frequently associated with malignancy (P = 0.042). Factors that significantly worsened event-free survival were age at diagnosis earlier than 112 months (P = 0.0035) and duration of symptoms lasting more than 2 months (P = 0.0029)., Conclusion: The results of this international study provide important information regarding the clinical presentation, diagnostic workup, and treatment of PTTs in children, casting new light on the biological behavior of PTTs to ensure appropriate treatments., Level of Evidence: NA Laryngoscope, 130:E243-E251, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Background: Endotracheal and endobronchial metastases (EEM) is a rare manifestation in primary lung cancer. It has not yet been reported in Chinese literatures. The aim of this study was to summarize and analyze the clinical feature of lung cancer with EEM., Methods: We retrospectively reviewed 6 patients who presented with EEM of lung cancer from Peking University Third Hospital from January 2015 to December 2018. With "endotracheal metastases, endobronchial metastases, lung cancer" as the keywords, 13 cases were retrieved from PubMed database until February 2020. The clinical, radiologic and bronchoscopic data were collected., Results: Six patients were selected from 967 patients with lung cancer, and all were diagnosed with lung cancer and EEM simultaneously. There were 4 cases of squamous cell carcinoma, 1 case of adenocarcinoma, and 1 case of small cell lung cancer. One patient had stage IIIb and 5 patients had stage IV. There were 5 cases of central lung cancer and 1 case of peripheral lung cancer. EEM on bronchoscope examination presented as endoluminal nodular or polypoid lesion in 5 patients, and abnormal white bulge in 1 patient. 5 cases metastasized to the contralateral bronchus, 1 case to the ipsilateral bronchus and 1 case to the trachea. The median overall survival was 7.5 months. Totally 13 cases of lung cancer with EEM were retrieved from PubMed database. 12 cases were diagnosed during the follow up after lung cancer resection. There were 8 cases of squamous cell carcinoma and 9 cases of central type. Endotracheal or endobronchial nodules showed in 10 cases and eccentric wall thickening in 2 cases were seen on chest computed tomography (CT), which corresponding to the nodular or polypoid lesion bronchoscopically. 5 cases metastasized to the contralateral bronchus, 10 cases to the trachea and 1 case to the ipsilateral bronchus., Conclusions: EEM is a rare metastasis of lung cancer, which can occur at the initial diagnosis of lung cancer or after surgical resection. It is often seen in the patients of squamous cell carcinoma with central type in advanced stage. The prognosis is poor.