Treatment outcomes of patients with primary tracheal tumors - analysis of a large retrospective series.

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.

Comparative long-term outcomes of airway resection and functional reconstruction for papillary thyroid cancer.

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.

Tracheobronchial Adenoid Cystic Carcinoma Treated Successfully With Chemoradiotherapy Followed by Durvalumab: A Case Report.

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.

Clinicopathological characteristics of patients with primary tracheal tumors: Analysis of eighty-nine cases.

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.

[Tracheal Tumours]. / Trachealtumoren.

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.

Successful endotracheal intervention for primary tracheal acinic cell carcinoma: A case report and literature review.

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.

Benign cervical schwannoma with tracheal invasion.

Schwannomas commonly develop in the cervical region, 25% - 45% of cases are diagnosed in this anatomical region. Tracheal neurogenic tumors are exceedingly rare and can be misdiagnosed as invasive thyroid carcinomas or other infiltrating malignancies when present at the level of the thyroid gland. Here, we present a case of synchronous benign cervical schwannoma with tracheal invasion and papillary thyroid carcinoma in a patient who was initially hospitalized for COVID-19. The patient presented with dyspnea that was later found to be caused by tracheal extension of a cervical tumor. Surgical excision was performed, and the surgical team proceeded with segmental tracheal resection, removal of the cervical mass, and total thyroidectomy. The specimen was sent for pathological analysis, which revealed synchronous findings of a benign cervical schwannoma with tracheal invasion and papillary thyroid carcinoma. The literature on this subject, together with the present case report, suggests that neurogenic tumors should be included in the differential diagnosis of obstructing tracheal cervical masses. Surgical excision is the first-line of treatment for benign cervical schwannomas.

Molecular Biology and Therapeutic Targets of Primitive Tracheal Tumors: Focus on Tumors Derived by Salivary Glands and Squamous Cell Carcinoma.

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.

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.

Successful Treatment with Definitive Concurrent Chemoradiotherapy Followed by Durvalumab Maintenance Therapy in a Patient with Tracheal Adenoid Cystic Carcinoma.

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.

Retrospective study of outcomes after extended resection for tracheobronchial adenoid cystic 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.

Airway and Hemodynamic Considerations for the Anesthetic Management of an Intraluminal Tracheal Plasmacytoma.

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.