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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.)

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.)

Diseases of the trachea are rare, while other diseases associated with shortness of breath and pulmonary symptoms are common, which is why the necessary diagnosis and therapy are regularly delayed. At the same time, diseases range from simple scarring stenosis to extensive tumour disease. Patients may be otherwise healthy, suffer from autoimmune disease, or have severe general illnesses, as in tracheotomised patients. Therefore, history and clinical examination provide very important clues to a disease of the trachea. Subsequent diagnostics should be ordered in a reasonable manner and lead to the correct diagnosis and subsequent therapy in a timely manner. Functional, radiological and endoscopic examinations, each with a special focus in experienced hands, avoid duplicate examinations or misinterpretations. Patients are grateful because diseases of the trachea - a central airway that cannot be bypassed - are experienced as life-threatening or at least as severely limiting the quality of life., 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.)

Schwannoma in primary neurogenic tumors of the trachea is an extremely rare disease. A 21-year-old male patient with stridor was followed up with a diagnosis of asthma for two years. While no lesion was observed in the chest X-ray, tracheal schwannoma was diagnosed in the case who underwent tracheotomy due to the lesion obstructing the trachea almost completely on chest CT. The clinical findings of the patient improved ultimately in the postoperative period. We aimed to discuss the approach and follow-up of the disease with our case, although it is rare that schwannoma might be in the differential diagnosis of masses with upper airway obstruction.

Primary tracheal schwannomas are rare benign tumours. This is a case report, and therefore, no specific methods or results are applicable. We here report a case of a tracheal schwannoma in an early adolescent girl presenting with subcutaneous emphysema and symptoms of airway obstruction. Tracheal resection and reconstruction by primary anastomosis were performed. Pathology confirmed the diagnosis of tracheal schwannoma. This is an unusual life-threatening presentation of a benign rare tracheal tumour with a challenging approach to management., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Lymphoma can appear in all parts of the body and present with different symptoms. However, bronchial lymphoma is rare and can be misdiagnosed as airway malignancy or lung disease.Patient: An older adult woman with tracheal lymphoma experienced severe breathing difficulties, and chest computed tomography indicated severe narrowing of the airway. She did not respond to repeated antibiotic treatment, and she was eventually diagnosed with lymphoma based on pathology after surgical removal of the tumor., Diagnosis: The patient received a diagnosis of thoracic tracheal stenosis due to intratracheal inflammatory granulomatous lesions or a tumor., Interventions: Treatment involved the use of a high-frequency electrotome, freezing, and argon plasma coagulation., Outcomes: The patient reported improvements in dyspnea, cough, and other symptoms after the operation. The pathological results confirmed follicular lymphoma. Reexamination by fiberbronchoscopy indicated that the degree of stenosis in the middle and upper tracheal segments was significantly reduced following interventional therapy., Conclusion: Endoscopic interventional therapy can be an effective treatment for tracheal lymphoma., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

A 41-year-old male presented to the Department of Pulmonary Medicine, with shortness of breath for 1 year. Though on treatment for bronchial asthma, he was not responding. The chest radiograph was normal (Fig. 1). On spirometric examination, the flow-volume curve showed flattening of the expiratory limb, suggesting variable intrathoracic obstruction. Fiber-optic bronchoscopy was, hence, done and it revealed a growth in the trachea (Fig. 2). Biopsy was deferred due to the risk of bleeding. Computed tomography (CT) of the chest also showed tracheal growth (Fig. 3). The patient was planned for rigid bronchoscopy. Meanwhile, the patient presented with expectoration of a piece of that growth. Histopathological examination revealed an adenoid cystic carcinoma (Fig. 4)., (© Journal of the Association of Physicians of India 2023.)

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.

A 34-year-old pregnant woman in the 34th week of gestation with uncontrolled asthma was admitted because of asthma exacerbation. Although she received bronchodilators and systemic corticosteroids, respiratory failure rapidly progressed. Chest computed tomography revealed a mass occluding approximately 80% of the tracheal lumen. After urgent Caesarean section, endobronchial resection was performed. The pathological findings of the resected tumor were compatible with tracheal glomus tumor. Tracheal tumors are often misdiagnosed as asthma, but its complication with asthma is rare. Even if the diagnosis of asthma is definitive, clinicians should consider coexisting diseases, including tracheal tumors, when asthma control is poor.

Objectives: The incidence of tracheal cancer is low, few clinicians get much experience and the awareness may be low. Recent data on the treatment and outcome are limited. The aim of the present study was to present updated, national data on the incidence, characteristics, treatment and outcome for patients with tracheal cancer., Methods: All tracheal cancers registered at the Cancer Registry of Norway in 2000-2020 were extracted. The patient and tumour characteristics age, sex, stage, histology and treatment modality (surgery and radiotherapy) were examined. Overall, median and relative survival were estimated. Cox regression models were used to identify independent prognostic factors., Results: The 77 patients diagnosed with tracheal cancer equals a crude incidence rate and an age-standardized incidence rate of 0.075 and 0.046 per 100,000 per year respectively. The mean age was 63.8 years (range: 26-94). The numerical preponderance of men (n = 41) is not statistically significant. Eighteen patients (23.4%) were diagnosed in the localized stage. The 5-year overall survival was 31.7% [95% confidence interval (CI): 21.0-42.9], and in those treated with surgical resection or curative radiotherapy, it was 53.7% (95% CI: 26.1-75.0) and 37.8% (95% CI: 18.8-56.7), respectively. Age, histological type and treatment modality were identified as independent prognostic factors., Conclusions: Despite improved survival, the prognosis for patients with tracheal cancer is still poor. Few are diagnosed in the early stage and thus most are not eligible for curative treatment, mainly surgery. An increased awareness and diagnosis in the earlier stage is crucial., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

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).)

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.)

Tracheal tumors are rare, accounting for 0.1% of all malignancies. Squamous cell carcinoma and adenoid cystic carcinoma are the two most prevalent tracheal cancers. Less than 20 cases of extramedullary plasmacytoma in the trachea and main bronchus have ever been documented in the literature, making it extremely uncommon. Although the origin of these lesions is unclear, viral pathogenesis and persistent inflammation are thought to be the main causes. Clinically, these individuals exhibit vague symptoms such as stridor, a persistent cough, dyspnea, or wheezing, making a correct diagnosis difficult., (© 2023 The Authors. Thoracic Cancer published by China Lung Oncology Group and John Wiley & Sons Australia, Ltd.)

Background: Tracheal chondrosarcoma is an extremely rare, slow-growing, malignant tumour. This study aims to analyze the cases of tracheal chondrosarcoma published in the literature and our case report, in order to better define tracheal chondrosarcoma management. Methods: A systematic review of the English literature was carried out for fully described tracheal chondrosarcoma cases. Additionally, we reported a new case of a 58-year-old man undergoing tracheal resection and reconstruction for tracheal chondrosarcoma. Results: To date, 30 cases were published. This tumour predominantly involved male patients (93%; median age: 65 years), generally conditioning dyspnoea and cough. Most of the patients underwent tracheal resection with end-to-end anastomosis, without recurrence (median follow-up: 2 years). Tumours endoscopically treated recurred in half cases. Conclusion: Tracheal resection is the treatment of choice for chondrosarcoma, with an excellent prognosis. Endoscopic treatment and/or radiotherapy should be indicated for patients unfit for surgery.

Primary adenoid cystic carcinoma (ACC) of the trachea is a rare entity, with a 5-year survival between 50% and 80% for resectable cases and 30% in case of unresectable disease. We report a case of a primary ACC on a woman in her 70s that presented with a drawn-out history of dyspnoea. She was diagnosed with an unresectable obstructive tumour of the trachea, which required the placement of a Y-shaped stent. The patient underwent concomitant chemoradiotherapy, with partial response, and is still in follow-up, without evidence of disease progression., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To determine the main forms of primary tracheal cancer (PTC), to specify the indications for various surgeries in these patients depending on extent and localization of lesion., Material and Methods: There were 263 PTC patients. Benign tumors were diagnosed in 68 (25.9%) patients, malignancies - in 195 (74.1%) cases. Tracheal cancer includes 3 basic morphological variants - adenocystic cancer (49.7%), carcinoid (18.7%) and squamous cell carcinoma (19.0%). Other forms of malignancies were much less common. We applied endoscopic intraluminal and open surgeries. In malignant PTC, open surgeries were performed in 165 (84.6%) out of 195 patients. Baseline palliative endoscopic treatment was performed in 30 patients. They underwent airway recanalization (with subsequent tracheal stenting in 19 patients). Endoscopic resection was preferred for benign tumors., Results: Twenty (12.1%) patients died after open surgery, and 1 (3.3%) patient died after endoscopic procedure. Most lethal outcomes occurred in early years of development of tracheal surgery. The causes of mortality were tracheal anastomotic failure in 12 patients, pneumonia in 6 patients, and arterial bleeding in 2 patients. Severe postoperative period was observed in all 3 patients after tracheal replacement with a silicone prosthesis. Long-term treatment outcomes depended on morphological structure of PTC. Favorable results were observed in patients with neuroendocrine tumor (carcinoid), worse outcomes in adenocystic cancer and unfavorable results in squamous cell carcinoma ( p <0.0013). Five-year survival rates were 75%, 65.6%, and 13.3%; 10-year survival rates were 75%, 56.2%, and 13.3%, respectively. These outcomes after combined treatment of primary tracheal cancer were significantly better compared to lung cancer ( p <0.05 when compared to global data)., Conclusion: Treatment of primary tracheal cancer should be based on classical principles of modern oncology (combined therapy, tumor resection with lymphadenectomy). Open and endoscopic interventions are justified. PTC is characterized by more favorable outcomes compared to lung cancer. It is difficult to analyze long-term results in tracheal cancer depending on various features of tumor process due to small number of observations. Accurate conclusions require multiple-center studies, preferably with international participation, which can convincingly prove certain concept.

Introduction: Tracheal tumors are rare, they are most often malignant and can manifest themselves by a non-specific respiratory symptomatology with progressively increasing dyspnea orienting in the first place towards a COPD or even an asthma. Among them, tracheal lipoma is exceptional. Its management is based on removal by rigid bronchoscopy., Observation: We report the case of a 73-year-old male patient who presented with non-specific dyspnea that progressively worsened over several months. The EFR showed a flattening of the flow-volume curves, the CT scan showed an anterolateral oval tracheal tumor with fatty density, the bronchial endoscopy showed a tumor lesion with stenosis of about 90% of the airway. Management consisted of a rigid bronchoscopy to delete obtruction with biopsies. Anatomopathology concluded to a tracheal lipoma., Conclusion: Progressively worsening dyspnea, especially if there are signs of inspiratory dyspnea, required a systematic bronchial endoscopy to avoid the possibility of a tracheal tumor., (Copyright © 2021 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

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.

Glomus tumors (GTs) are uncommon benign tumors, accounting for <2% of all soft tissue tumors and usually occur within the dermis or subcutis of distal extremities. Primary tracheal GT is rare, and to date less than 40 tracheal GTs have been reported. Tracheal GT usually present as a polypoidal mass. They express smooth muscle markers, and are negative for cytokeratin (CK) and neuroendocrine markers on immunohistochemistry (IHC). We here report a case of tracheal GT showing diffuse strong aberrant immunoexpression for synaptophysin, initially construed as carcinoid. Focal synaptophysin expression has been described in few gastric GTs, and a nasal GT. Diligent histomorphological examination and careful selection of IHC panel helps in clinching the diagnosis. Complete resection is the standard treatment of modality., (© 2020. Springer Science+Business Media, LLC, part of Springer Nature.)

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.

Tracheal tumors or masses causing critical airway obstruction require resection for symptom relief. However, the location and extent of these tumors or masses often preclude conventional general anesthesia and tracheal intubation. Peripheral cardiopulmonary bypass often is required before anesthetizing these patients. Herein, two cases of patients with tracheal masses, in whom awake peripheral cardiopulmonary bypass was instituted, are reported. The first case was that of an obese male child weighing 102 kg, with tracheal rhinoscleroma, who developed Harlequin, or north-south, syndrome after institution of femorofemoral venoarterial partial cardiopulmonary bypass. The second case was that of a female patient with adenoid cystic carcinoma of the trachea causing near-total central airway occlusion. She had severe pulmonary artery hypertension, which prevented the use of venovenous bypass. Instead, femoral vein-axillary artery venoarterial bypass was established to avoid Harlequin syndrome. Some of the challenges encountered were the development of Harlequin syndrome with risk of myocardial and cerebral ischemia, type and conduct of extracorporeal bypass, choice of monitoring sites, and provision of regional anesthesia for peripheral extracorporeal cannulations. Management of such patients needs frequent troubleshooting and multidisciplinary coordination for a successful surgical outcome., (Copyright © 2020 Elsevier Inc. All rights reserved.)

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.)

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.)

A 66-year-old male patient who had received chemoradiotherapy (CRT) for laryngeal cancer 2 years ago visited a local doctor complaining of dyspnoea and wheezing. CT scan showed narrowing of the trachea caused by a tumour. We intubated the trachea over the tumour using a bronchoscope. A week later, the truncated tracheal tumour obstructed the tracheal tube, compromising the patient's breathing. We removed the obstructed tube and inserted a new one. We submitted the tissue from the tube to a pathologist. Histopathological diagnosis was pleomorphic carcinoma, a subtype of sarcomatoid carcinoma. The mechanism of epithelial-mesenchymal transition (EMT) occurring after CRT was detected in the tumour. Because he had undergone CRT for laryngeal cancer, surgery was not indicated, and we started radiation therapy. Sarcomatoid carcinomas including pleomorphic carcinoma of the trachea are extremely rare, with few reported cases, and EMT is associated with this histological type and CRT., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Critical central airway obstruction has always been a dreaded complication to which interventional pulmonologist commonly encounters. There have been various modalities which are used for the management and palliation, which includes mechanical coring, laser, cryoextraction, electrocautery and airway stenting. Rigid bronchoscopy with or without jet ventilation has been corner stone of therapeutics and palliation of central airway obstruction. There are only a few conditions where it is not possible to use rigid bronchoscopy. Here we report a case of metastatic tracheal tumour which presented with critical airway obstruction in a patient who had atlantoaxial instability (AAI) due to rheumatoid arthritis. Here we used endobronchial ultrasound scope (EBUS) via esophageal route, i.e. EUS-B guided approach for sampling of the tracheal tumour, and intratumoral chemotherapy was instilled in multiple sessions, which resulted in shrinking of tumour, thus relieving the critical airway obstruction. This is the first report of using EUS-B approach for intratumoral chemotherapy for tracheal tumors. Bronchoscopic intratumoral chemotherapy therapy (BITC) in tracheal tumors is also one of the options but has not been explored much and there has been a dearth of literature for it.

Objective: This study aimed to determine the sonographic features suggestive of extrathyroidal extension (ETE) of thyroid cancers., Materials and Methods: We retrospectively reviewed the sonographic images of 1656 consecutive patients who had undergone thyroidectomy in 2017. The diagnostic performance of sonographic features suggestive of ETE was evaluated using operation and histopathologic reports. Sonographic features for gross ETE to the strap muscle and minor ETE were assessed for thyroid cancer abutting the anterolateral thyroid capsule. Sonographic features for tracheal invasion were assessed according to whether the angle between the tumor and the trachea was an acute, right, or obtuse angle. Sonographic features for recurrent laryngeal nerve (RLN) invasion were assessed based on the association between the tumor and tracheoesophageal groove (TEG) as preserved normal tissue, abutting or protruding into the TEG., Results: ETE was observed in 783 patients (47.3%), including 123 patients with gross ETE (7.4% [strap muscle, n = 97; RLN, n = 24; and trachea, n = 14]) and 660 patients with minor ETE (39.9%). Regarding the diagnosis of gross and minor ETE to the strap muscle, sonographic features of replacement of the strap muscle and capsular disruption showed the highest positive predictive value (75.9% and 58.5%, respectively). Thyroid cancer forming an obtuse angle with the trachea had the highest sensitivity for the diagnosis of tracheal invasion (85.7%), and thyroid cancer protrusion into the TEG showed the highest sensitivity for the diagnosis of RLN (83.3%)., Conclusion: Sonography is considered beneficial in the diagnosis of ETE to the strap muscle, trachea, and RLN. Assessment of ETE is important for the accurate staging of thyroid cancer, which in turn determines the extent of surgery or whether active surveillance is appropriate or not., Competing Interests: The authors have no potential conflicts of interest to disclose., (Copyright © 2020 The Korean Society of Radiology.)

Evidence on survival of malignant mesothelioma (MM) and other rare thoracic cancers is limited due to the rarity of these cancer sites. Here, we provide a comprehensive overview of MM incidence and survival after MM and other rare thoracic cancers in Germany and the United States (US). Incidence was estimated from a German National Cancer Database and from the Surveillance, Epidemiology and End Results (SEER) 18 database for 2000-2014. Patients diagnosed in 1997-2013 with malignant epithelial tumors of the trachea (Etra), epithelial tumors of the thymus (Ethy) and MM were extracted from a German cancer survival database and from the SEER 13 database. Period analysis was employed to compute 5-year relative survival (RS). During 2000-2014, an annual average of 0.9 and 0.6 MM cases per 100,000 person-years was diagnosed in Germany and the US. Rates decreased in Germany and in the US. Patients with Ethy had highest 5-year RS with US patients surviving longer (69.1% compared to 63.7%, p = 0.02). Survival after Etra was comparable in both countries (Germany 33.6%, US 34.4%, p = 0.07). Survival in MM patients was poor overall (Germany 11.8%, US 12.1%, p < 0.01). Survival improvements were only observed in MM patients in Germany (10.8% [2002-2007] vs. 13.0% [2008-2013], p < 0.01). The lack of progress in survival for Etra and Ethy patients underlines the need of novel preventive, therapeutic and diagnostic approaches. MM incidence significantly decreased in Germany and in the US. Further monitoring of MM incidence is warranted given that a peak in incidence is expected in 2020-2030 in Western countries., (© 2020 The Authors. International Journal of Cancer published by John Wiley & Sons Ltd on behalf of UICC.)

Background: Malignant peripheral nerve sheath tumours (MPNSTs) of the trachea are extremely uncommon neoplasms with unknown genetic and clinical profiles. Only individual cases have been reported in the literature to date., Case Presentation: Here, we present a rare case of a 61-year-old female patient with a primary MPNST of the trachea who complained of irritating cough and progressively increasing breathlessness for 4 weeks. This patient initially underwent intraluminal resection of the mass and was misdiagnosed with clear cell sarcoma. Less than a year later, the mass relapsed, and the obstructive symptoms reappeared and gradually worsened. Debulking of the endotracheal tumour mass was performed once again, and an MPNST was definitively diagnosed. Open sleeve tracheal resection and tracheoplasty were later performed with curative intent. This patient was alive without recurrence at her six-month postoperative follow-up. We also compared the clinical outcomes of previously reported cases of MPNSTs and our case., Conclusions: This paper emphasizes that thoracic surgeons should be aware that malignant peripheral nerve sheath tumours of the trachea can be misdiagnosed in clinical practice and must be included in the differential diagnosis of tracheal neoplasms.

Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht.

A 17-year-old, female cockatiel ( Nymphicus hollandicus ) was presented for a 1-week history of respiratory distress. The clinical signs were consistent with tracheal obstruction; therefore, an air sac cannula was placed to reduce respiratory compromise. Radiographic images of the patient revealed a round, intraluminal, soft tissue opacity in the trachea. The obstruction could be visualized via transtracheal illumination, but tracheoscopy was not feasible because of the size of the patient. Attempts to manually extirpate the obstruction were unsuccessful, and the trachea subsequently avulsed in the midcervical region. Tracheal resection and anastomosis was performed, and the obstruction was removed with the associated tracheal ring. The patient recovered uneventfully and was doing well at follow-up evaluations at 9 days, 24 days, and 10 months after the surgical procedure. The histologic diagnosis was ossifying myelolipoma arising from bone marrow within the tracheal ring.

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.

Tracheal squamous cell carcinoma is the most common pathology in smokers while ACC is more prevalent among non-smokers. These tumors tend also to be diagnosed late on account of delayed specific symptoms as hemoptysis, dyspnea, cough, hoarseness, and stridor being the most common. Management of tracheal tumors is essentially multidisciplinary. It includes interventional endoscopy, surgery, radiotherapy, and/or end luminal brachytherapy. Extensive segmental resection of the trachea is the potentially curative treatment of choice for primary lesion. The sleeve trachea resection is one of the optimal surgical modalities. The other options are: partial tracheal wall resection and immediate tracheal reconstruction, total laryngectomy plus partial resection of trachea and primary reconstruction, laryngeo-tracheal resection, cervico-mediastinal exenteration, or carinal resection and reconstruction. Trachea anastomosis is suitable for small defects. The platysma myocutaneous flap combined with the facial flap of the sternohyoid muscle, sternocleidomastoid myoperiosteal flap and the pectoralis major musculocutaneous flap are applied to reconstruct the defects of cervical trachea. Post-operative radiation therapy, in many cases, is considered a fundamental part of treatment. Contraindications to surgery include: metastatic disease, invasion of adjacent organs, involvement of airway greater than could be safely resected (i.e. >50% of trachea), involvement of airway that would leave grossly positive margins after resection, spinal kyphosis, or poor medical condition of the patient. Patients in the current report tolerated therapy well with the use of modern RT techniques and dose delivery to 60 to 64 Gy to a large extent of the central airway. Additional data and meta-analysis are required to validate the efficacy of chemoradiation in comparison to primary RT alone for unresectable cases and subsequently identify improved systemic therapies. Further investigation into the potential role of additional therapies, such as adjuvant chemotherapy or immunotherapy, may be worth exploring. Our initial findings suggest the use of concurrent chemotherapy in addition to RT in patients with locally advanced tracheal SCC.

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.)

Primary chondrosarcoma of the trachea is an extremely rare tumor. We report two cases of tracheal chondrosarcoma describing the role of surgical and conservative treatment. Endoscopic treatment with rigid bronchoscopy was performed in both patients to restore airway patency and obtain histological specimens for diagnosis. One of the patients subsequently underwent successful tracheal resection and reconstruction. The other patient, who had a contraindication to surgical treatment due to associated diseases underwent iterative endoscopic LASER treatment and is alive three years after the first diagnosis. Surgical treatment remains the treatment of choice of tracheal chondrosarcoma. When surgery is contraindicated endoscopic treatment may allow relatively longterm survival due to the slow growth of these tumors.

Introduction: Our purpose is to evaluate our results of surgery for tracheobronchial carcinoid tumour as well as the long-term survival., Methods: This is a retrospective and descriptive study performed in the department of thoracic surgery of CHU Hassan II (Marocco) over a period of 9 years. It concerns all patients with a tracheal or bronchial carcinoid tumour who underwent surgery., Results: Twenty-three patients with a mean age of 39 years were operated on for 24 carcinoid tumours. The sex ratio was 0.29. The diagnostic delay ranged from 3 months to 8 years and the main symptom was haemoptysis in 74% of cases (n=17). The tumour was localized in the right bronchial tree in 70% of cases (n=16). The procedures performed were tracheal resection and end-to-end anastomosis in 1 case, lobectomy in 12 cases including 3 sleeve lobectomies, bilobectomy of middle and lower lobes in 7 cases and pneumonectomy in 4 cases. The prognosis was favourable in 91% after an average follow-up of 29 months., Conclusions: Surgery remains the only curative therapeutic option for tracheobronchial carcinoid tumours with acceptable morbidity and mortality., (Copyright © 2019 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Background: The incidence of primary tracheal tumors is very low. Tracheal synovial sarcoma (SS) is even an extremely rare entity. Diagnosis of tracheal SS can be achieved with chromosomal translocation studies along with immunohistochemistry. Margin-free resection is the gold standard treatment., Case Presentation: We report a case of tracheal SS, which presented with stridor with a history of chronic cough and was diagnosed with a battery of clinical investigations and managed successfully with tracheal resection surgery. In histology, it may mimic Ewing's sarcoma. Immunohistochemically, SS stains positive for cytokeratin, epithelial membrane antigen, vimentin, and S100. Chromosomal translocation t(X;18) (p11;q11) is found in almost all SS. This genetic signature is the gold standard diagnostic modality for these tumors., Conclusion: Diagnosis of tracheal synovial sarcoma is challenging because of the rarity of the disease and common presenting symptoms to other tracheal pathology and is supplemented with chromosomal translocation study along with histopathology and immunohistochemistry. Tumor coring before definite surgical resection facilitates lung perfusion in obstructive tracheal pathology. A multidisciplinary team approach for diagnosis and management along with long-term follow-up are warranted.

Oesophago-pleural fistula is an uncommon complication after pneumonectomy, usually related to high morbidity and mortality. Due to its rarity and heterogeneous clinical presentation, its diagnosis and management are challenging issues. Here, we report the case of a patient with a history of pneumonectomy for a tracheal tumour, who developed an asymptomatic oesophago-pleural fistula 7 years after primary surgery. In consideration of the patient's good clinical status and after verifying the preservation of respiratory and digestive functions, a bold conservative approach was adopted. Five-year follow-up computed tomography did not disclose any sign of recurrence of disease and showed a stable, chronic fistula., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

A rare clinical observation of primary tracheal MALT lymphoma is reported and difficulties of differential diagnosis are discussed. Tracheal neoplasms are rare tumors and characterized by delayed diagnosis after clinical manifestation (tracheal stenosis and associated complications). These tumors often occur an advanced age patients that complicates examination and surgical treatment. High risk is determined by type of surgery, possible postoperative complications and senile age.

Rationale: Primary schwannoma is extremely rare in the trachea, and its optimal treatment has not yet been established. Previous literature have indicated that traditional resection by thoracotomy is an effective surgical procedure but with huge trauma, and endoscopic excision is a minimally invasive surgical method but with possibility of recurrence. Window resection was usually utilized for selected patients with trachea invasion by thyroid carcinoma, but video-assisted thoracoscopic window resection for trachea schwannoma has not been reported previously., Patient Concerns: A 23-year-old woman was admitted to hospital due to dyspnea, coughing and wheezing that had persisted for 2 months with aggravation for 1 week., Diagnoses: Chest computed tomography (CT) scan revealed a well-circumscribed soft-tissue mass located on the right lateral posterior wall of the trachea. Bronchofibroscopy (BFS) showed a whitish, smooth and round mass with a wide base in the trachea. Immunohistochemical staining demonstrated cells labeled with Vim (+), S-100 (+), SOX-10 (+), SMA (-), CK (-). Histopathological examinations showed that the mass was a schwannoma., Interventions: The tumor was nearly completely excised via BFS, but relapsed 2 times at 12 days and 3 weeks after endoscopic resection. Finally, the patient underwent video-assisted thoracoscopic window resection of trachea., Outcomes: The patient recovered rapidly and no recurrence was observed over 6 months of follow-up., Lessons: The treatment of tracheal schwannoma depends on the characteristics of tumor and the condition of patient. Surgical resection is a preferred alternative for sessile or transmural tumors and recurrence after endoscopic excision. Tracheal window resection by video-assisted thoracoscopy is beneficial for some appropriate patients with a small and sessile tumor.