Tracheal Schwannoma Presenting as Subcutaneous Emphysema and Pneumomediastinum.

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.

Interventional treatment of giant tracheal lymphoma under rigid bronchoscopy: A case report and literature review.

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.

The Novel Application of Robotic-Assisted Bronchoscopy Combined with Photodynamic Therapy for Adenoid Cystic Carcinoma of the Trachea.

Robotic platforms have been widely used in the various fields of clinical diagnosis and therapy of diseases in the past decade. Robotic-assisted bronchoscopy (RAB) demonstrates its advantages of visibility, flexibility, and stability in comparison to conventional bronchoscopic techniques. Improving diagnostic yield and navigation yield for peripheral pulmonary lesions has been defined; however, RAB platform of treatment was not reported. In this article, we report a case of a 52-year-old woman who was diagnosed with the tracheal adenoid cystic carcinoma and recurred in the second postoperative year, leading to the involvement of the entire tracheal wall and lumen obstruction. Since the lesion was inoperable, we combined RAB and photodynamic therapy (PDT) for the patient. The potential advantages of using RAB for PDT delivery include precise light irradiation of target lesions and stable intra-operative control over the long term. This is a novel application of RAB combined with PDT for airway diseases. The case report may provide a new insight into the diagnosis and treatment of pulmonary diseases. In addition to improving the diagnostic rates, the RAB platform may also play an important role in the treatment of airway and lung disease in the future.

An extremely rare tracheal tumor in coronavirus disease-2019 screening: Marginal zone lymphoma.

Mucosal-associated lymphoid tissue lymphoma is extremely rare due to the scarcity of lymphoid tissue in the trachea. To date, approximately 20 cases of tracheal mucosa-associated lymphoid tissue lymphoma have been reported. This case report presents a primary tracheal extranodal marginal zone lymphoma case detected incidentally during the coronavirus disease-2019 screening.

Tracheal cancer: a rare and deadly but potentially curable disease that also affects younger people.

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.

Endoscopic Excision of Primary Tracheal Schwannoma: A Case Report.

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.

Malignant transformation of a solitary tracheal papilloma in the absence of human papillomavirus infection in a patient with 48, XYYY syndrome: case report.

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.

Proton Beam Therapy as a Curative Treatment for a Young Case of Unresectable Tracheal Adenoid Cystic Carcinoma.

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.

Primary acinic cell carcinoma of the trachea: A case report and literature review.

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.

Solitary extramedullary plasmacytoma of the left main bronchus: A case report and literature review.

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.

Tracheal Glomus Tumor Complicated with Asthma Exacerbation in a Pregnant Woman.

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.

[Surgery of primary tracheal tumors]. / Khirurgiya pervichnykh opukholei trakhei.

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.

Primary adenoid cystic carcinoma of the trachea: an elusive diagnosis of chronic dyspnoea.

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.

Tracheal resection and anastomosis for squamous cell carcinoma.

Tracheal malignant tumors are uncommon lesions. The rarity of this condition may generate uncertainties in the diagnosis and treatment. For this reason especially, the surgical treatment should be performed only in centers with a high expertise in tracheal surgery. If the involved tracheal tract is less than 4-5 cm and the tumor is localized, the treatment of choice is based on a segmental tracheal resection with an end-to-end anastomosis. In this video tutorial, we describe how we perform tracheal resection with an end-to-end anastomosis in a patient with a squamous cell carcinoma.

[Tracheal lipoma: An exceptional cause of tracheal tumors]. / Lipome trachéal : une cause exceptionnelle de tumeur trachéale.

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.

[Unexpected cause of tracheal obstruction: pleomorphic adenoma with lipometaplasia]. / Adenoma pleomorfo con lipometaplasia: inesperada causa de obstrucción traqueal.

Pleomorphic adenoma of the trachea is a benign tumor, extremely rare, that generates obstructive symptoms and sometimes is confused with asthma, which delays its diagnosis. We present the case of a 40-year-old woman with history of asthma and airway obstruction, of 8-months duration. Fibrobronchoscopy showed polypoid, pedunculated lesion in first tracheal ring which occluded 80% of the lumen, that was resected. The histopathological diagnosis was pleomorphic adenoma with lipometaplasia. Treatment of these tumors consists in complete resection of the lesion and long-term follow-up due to low probability of recurrence, malignancy and metastasis.

El adenoma pleomorfo de tráquea es un tumor benigno, infrecuente, que genera cuadros obstructivos, pudiendo confundirse con asma, lo que retrasa su diagnóstico. Presentamos el caso de una mujer de 40 años con antecedentes de asma y episodios de obstrucción respiratoria de 8 meses de evolución. La fibrobroncoscopía mostró una lesión polipoide, pediculada, en el primer anillo traqueal, con oclusión de 80% de la luz, que se resecó. El diagnóstico histopatológico fue adenoma pleomorfo con lipometaplasia. El tratamiento de estos tumores consiste en la resección completa de la lesión y seguimiento a largo plazo por la baja probabilidad de recurrencia, malignización y metástasis.

Surgical management of tracheal chondrosarcoma.

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.

Compromiso tumoral de vía aérea en pediatría / Pediatric airway compromise by tumors

Los tumores traqueo bronquiales son extremadamente infrecuentes en la edad pediátrica e incluyen lesiones benignas y malignas. Por la baja frecuencia en niños y sintomatología respiratoria inespecífica, la sospecha diagnostica es habitualmente tardía. El tratamiento de elección en la mayoría de ellos es la resección quirúrgica abierta, sin embargo, la remoción endoscopia podría estar indicada es casos muy seleccionados con histología benigna y de localización accesible.

Tracheobronchial tumors are extremely rare in children and include benign and malignant lesions. Due to the low frequency in children and nonspecific respiratory symptoms, diagnostic suspicion is usually late. The treatment of choice in most of them is open surgical resection, however, endoscopy removal could be indicated in highly selected cases with benign histology and accessible location.

Tracheo-oesophageal groove Hodgkin's lymphoma presenting as stridor: a diagnostic challenge.

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.

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

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