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.

[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.

Adenoid cystic carcinoma of trachea: long-term disease control after endoscopic surgery and radiotherapy.

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.

Therapeutic strategy for tracheal chondrosarcoma: report of two cases.

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.

Tracheal tumors.

PURPOSE OF REVIEW: This article provides the most recent literature and experienced guidance about diagnosis and management of tracheal tumors, including future directions. RECENT FINDINGS: Tracheal tumors are challenging to diagnose given their nonspecific and late-onset symptoms. Critical airway obstruction is the most feared complication. The cornerstone in management of them is surgical resection whenever is possible. Different diagnostic tests (i.e., imaging and bronchoscopy) help in the assessment of resectability. Alternative management including interventional bronchoscopic techniques can be offered to patients who are not suitable for surgical resection. Radiation and chemotherapy can also be used as adjuvant, neoadjuvant or sole treatment in malignant tumors. Recent advances in tracheal transplantation and bioengineering with tracheal reconstruction have shown a new direction in the management of these tumors. All these topics will be discussed in this article. SUMMARY: Despite advances in diagnostic and therapeutic techniques, tracheal tumors are still a medical challenge. Although studies are still trying to find the best surgical techniques as well as the role for chemotherapy and radiation, most recent studies are addressing tracheal reconstruction and transplantation as the new horizon for management.

An Endotracheal Plasmablastic Lymphoma.

We describe an exceptionally rare case of a male patient with newly diagnosed advanced human immunodeficiency virus (HIV) infection, who presented with a plasmablastic lymphoma involving the right maxillary alveolar ridge with associated cervical lymphadenopathy. On a staging positron emission tomography computed tomography (PET-CT) scan, he was incidentally found to have an endotracheal tumour involving the anterolateral aspect of the mid-trachea. The tumour appeared to be well-vascularised at bronchoscopy and was confirmed as well-differentiated plasmablastic lymphoma. Plasmablastic lymphoma is a rare form of non-Hodgkin lymphoma and is associated with HIV. Tracheal involvement to the extent seen in our patient is exceptionally rare, and, to the best of our knowledge, has never been described.

Long-Term Outcome of Definitive Chemoradiotherapy and Induction Chemoradiotherapy Followed by Surgery for T4 Esophageal Cancer with Tracheobronchial Invasion.

BACKGROUND: T4 esophageal cancer (EC) that invades the trachea or bronchus often has poorer prognosis than other T4 ECs. We investigated the long-term results of definitive chemoradiotherapy (dCRT) or induction chemoradiotherapy followed by surgery (iCRT-S) in patients with T4 EC with tracheobronchial invasion (TBI). PATIENTS AND METHODS: From 2003 to 2013, 71 patients with T4 EC with TBI were treated in our institution; 58 underwent dCRT, and 13 underwent iCRT-S. The long-term results associated with survival were retrospectively analyzed, and prognostic factors were examined by univariable and multivariable analysis. RESULTS: The 1-, 2-, and 5-year overall survival for all patients with T4 EC with TBI treated by dCRT or iCRT-S was 57, 29, and 19%, respectively. Multivariable analysis revealed that clinical lymph node (LN) metastasis and the treatment period were significant prognostic factors. Clinical LN positivity had significantly poorer prognosis than LN negativity. The treatment outcome in the later period was significantly better than that in the earlier period. In particular, the outcome after dCRT revealed significantly better prognosis in the later compared with the earlier period, whereas the outcome after iCRT-S did not show such a difference. With respect to treatment modality, no significant difference in survival was observed between dCRT and iCRT-S. CONCLUSIONS: Clinical LN negativity and later treatment period were significantly good prognostic factors for T4 EC with TBI. The recent improvements in dCRT outcomes may help to achieve survival comparable to that of iCRT-S.

Survival trends in patients with tracheal carcinoma from 1973 to 2011.

PURPOSE: The prognosis for primary tracheal cancer is dismal. We investigated whether there has been improvement in survival in tracheal cancer patients and how treatment modality affected overall and cancer-specific survival. MATERIALS AND METHODS: Using the Surveillance, Epidemiology, and End Results database, 1144 patients with tracheal cancer were identified between 1973 and 2011. Patients were stratified by age group, gender, race, tumor histology, and treatment modality. Radical surgery and survival rates based upon these stratifications were determined. Longitudinal analyses of survival and the percentage of patients undergoing surgery and radiation were conducted. RESULTS: In the final cohort, 327 tracheal cancer patients (34%) underwent radical surgery. Patients of younger age, female gender, and who presented with non-squamous cell tumors were statistically more likely to undergo surgery. Over time, utilization of radiation has declined while use of radical surgery has increased. Concomitantly, 5-year survival has increased from approximately 25% in 1973 to 30% by 2006. Those who did not have surgery were 2.50 times more likely to die of tracheal cancer (95% Confidence Interval 2.00-3.11, p<0.001) than those who did have surgery. Additionally, patients who underwent radical surgery alone (without adjuvant radiation therapy) were 50% or 19% less likely to die of tracheal cancer than those who underwent no treatment or combination therapy, respectively (both p<0.001). CONCLUSIONS: Survival in patients with tracheal cancer is improving over time. The utilization of radical surgery is increasing and confers the highest survival advantage to patients who are candidates.

A 14-year-old boy with extranodal natural killer cell lymphoma of the nose, nasopharynx, larynx, and trachea in remission 6years after primary diagnosis. A longitudinal case report.

Nasal type extranodal natural killer/T-cell lymphoma (ENKTL) is a rare lymphoma in the USA and Europe but endemic in East Asia and in areas of South and Central America. Clinically natural killer cell lymphomas are divided into three categories; nasal, non-nasal and aggressive lymphoma/leukemia subtypes. ENKTL, nasal type occurs in the nose and can extend to the upper aero-digestive tract as reported in this longitudinal case study. This is a longitudinal report of progress of a 14-year-old boy with ENKTL originating in the nasal cavity with subsequent extension and recurrence in the contralateral nose, nasopharynx, larynx and trachea presenting with varying degrees of respiratory problems and eventually, respiratory distress. Caregiver refusal of stem cell transplantation prompted an alternative diagnostic and therapeutic approach. Clinical course with recurrences, extensions and remissions over 6years with tailored endoscopic surgical treatment and radiochemotherapy is documented to present a guide in the multidisciplinary management of this rare disease.

[Tracheal Resection and Primary Anastomosis for Adenoid Cystic Carcinoma Using an Extracorporeal Membrane Oxygenation].

We report a case of tracheal resection and primary anastomosis for adenoid cystic carcinoma using an extracorporeal membrane oxygenation (ECMO). A 45-year-old female was referred to our hospital because of a tracheal tumor that occupied most of the tracheal lumen. In case of airway obstruction by the tracheal tumor during anesthesia and operation, we decided to use ECMO before induction of general anesthesia. Under secure respiratory control using ECMO, tracheal resection and primary anastomosis was performed. Since histopathological examination revealed microscopically positive results at the surgical margin, postoperative adjuvant radiation therapy( 60 Gy/30 Fr) was conducted. Although a tracheal tumor is a relatively rare neoplasm, careful planning and a treatment strategy are necessary with special emphasis on the location and size of tumor. In this case, ECMO made a substantial contribution to secure respiratory control during surgery.

[Adenocystic carcinoma of the trachea]. / Adenokistoznyi rak trakhei.

It is presented 40-years experience of tracheal adenocystic carcinoma diagnosis and management in 144 patients. Peculiarities of clinical course and choice of diagnostic and therapeutic measures are discussed in relation to stenosis and hypoxia degree and severity of accompanying inflammatory complications of trachea, bronchi and lungs. Technical tools and original methods of resection and reconstruction of trachea and it's bifurcation after extended lung resection are described. Surgical management is carried out in 96 patients: all patients underwent circular resection of trachea (77) or it's bifurcation (19). Morbidity rate was 22.9% and mortality - 3.1%. Additional radiotherapy was carried out in 50 patients. 41 patients underwent radiotherapy alone. Radiotherapy improves remote results of management. 5-and 10-year survival rate was 78.3±6.1 and 45.9±7.9% after surgery alone, 92.0±3.9 and 77.1±6.5% after combined treatment and 76.4±6.5 and 55.0±10.7% after radiotherapy alone respectively.

Malignant tracheal tumors: a review of current diagnostic and management strategies.

PURPOSE OF REVIEW: This article reviews the current literature for the purpose of developing a practical approach for the diagnosis and management of primary tracheal tumors. RECENT FINDINGS: Because of nonspecific symptoms, tracheal tumors remain a diagnostic challenge. Currently available management strategies are not being optimally utilized due to lack of physician awareness and knowledge. The use of newer diagnostic modalities has increased diagnostic accuracy resulting in earlier detection in recent years. This review describes currently available diagnostic modalities along with relatively newer ones such as virtual bronchoscopy, anatomic Optical Coherence Tomography, spectroscopic techniques, and endobronchial ultrasonography. We will review and discuss management strategies including surgical options, adjuvant therapies, and interventional pulmonary techniques including their role in palliation. SUMMARY: Early detection along with improved surgical and interventional pulmonology techniques has led to a decline in the death rates from tracheal cancer in recent years. However, further studies are required to define the role of chemotherapeutic agents, combination therapies, and novel techniques such as tracheal transplantation, in the management of primary tracheal tumors. More robust evidence-based studies are needed to provide evidence for clinical practice guidelines for the treatment of primary tracheal tumors.

Primary tracheal adenoid cystic carcinoma: adjuvant treatment outcome.

BACKGROUND: Adenoid cystic carcinoma (ACC) is the second most common tracheal tumor, but its optimal treatment strategy is still controversial. METHODS: To further elucidate the impact of treatment on disease-free survival (DFS) and overall survival (OS), we retrospectively investigated different treatment modalities and outcomes of 56 patients with primary ACC of the trachea treated at four hospitals in Sichuan Province of China from 1995 to 2012. RESULTS: 52 patients were included in the analysis. 4 patients with unresectable tumors were treated primarily with radiotherapy (RT) alone. 11 of 48 patients who received surgery as primary therapy obtained complete resection without adjuvant therapy. 24 of 37 patients who had incomplete resection (R1, R2) received postoperative RT while 13 patients were treated without postoperative RT. Postoperative chemotherapy (CT) was used in 12 patients with postoperative RT. No significant difference was shown in DFS (p = 0.683) and OS (p = 0.829) between patients with complete resection and those with incomplete resection. Postoperative RT for patients with incomplete resection was associated with improved DFS (92 vs. 62 months, p = 0.027) and OS (125 vs. 78 months, p = 0.004). Postoperative chemotherapy (CT) following RT did not have a significant impact on DFS (p = 0.390) or OS (p = 0.646) in patients with positive margin. CONCLUSIONS: These observations suggest that postoperative RT should probably be recommended for patients with incomplete resection. Postoperative CT following RT in patients with incomplete resection did not seem to produce an additional survival benefit.

Primary fibrosarcoma of the trachea presenting with acute airway loss.

Primary sarcomas of the trachea are rare occurrences that present with nonspecific symptoms, making timely diagnosis difficult. We report a case of primary fibrosarcoma of the trachea that presented with acute airway loss secondary to tracheal discontinuity due to tumor destruction. This unusual clinical presentation highlights the difficulties posed in the diagnosis and management of tracheal sarcomas. A discussion of the relevant literature is presented.

[Treatment of neoplastic lesions of the central bronchi and trachea using endotracheobronchial surgery, intraluminal brachytherapy, combined radiotherapy and chemoradiotherapy].

There are summarized the foreign and domestic references of recent years devoted to methodology and the efficiency of the use of intraluminal high-dose radiation brachytherapy in patients with lesions of the central bronchi and trachea caused by primary and metastatic malignant tumors. It is presented own experience of applying this method in 207 patients. It is showed that in some patients to ensure the delivery of the radiation source to the area of interest it is advisable to perform firstly endotraheobronhial surgery with recanalization of the lumen of the respiratory pathways. The best is the use of intraluminal brachytherapy with high dose radiation. Palliative intraluminal irradiation of inoperable patients allowed achieving a good immediate results (65-95%), a significant reduction of the main symptoms--hemoptoe (87-95%), dyspnea (75-90%), obstructive pneumonia phenomena (50-85%), and significantly increasing survival median from 1-3 to 9-14 months. Following performance of chemoradiotherapy permitted increasing the survival median up to 15-20 months. The number of complications of intraluminal high-dose radiation brachytherapy was small, usually--pulmonary hemorrhage (2-7%) more likely developing when using large fractions--more than 10 g for 1 session.

Metallic stent placement for the management of tracheal carina strictures and fistulas: technical and clinical outcomes.

OBJECTIVE: The purpose of this article is to assess the technical and clinical outcomes of metallic stent placement in strictures and fistulas involving the carina. MATERIAL AND METHODS: We performed a retrospective analysis of patients who had undergone stenting for disease involving the carina. We initially reviewed the symptoms, underlying causes, and the types of stent configuration used. We also assessed the technical success rate of stenting, its effectiveness in achieving symptomatic relief, the incidence of stent-related complications, and stent patency. RESULTS: Thirty-two stenting procedures were performed in 23 patients (mean age, 56.3 years) for the treatment of strictures (n = 21), an esophagorespiratory fistula (n = 1), or both (n = 1) present in the carina. Three cases were associated with benign causes, whereas 20 were related to malignancies. Dyspnea was the most common symptom (n = 22). We placed metallic stents in four different configurations, among which placement in juxtacarinal segments was the most common configuration (n = 23). Technical success was achieved in 96.9% of cases, and symptomatic improvement was observed in 90.6% of cases. Stent-related complications were observed after 10 procedures (31.3%). Stent obstruction occurred in seven patients (21.9% of procedures), most commonly because of tumor progression. The mean follow-up period was 83.1 days, during which time 15 patients died as a result of disease progression, five were discharged without hope for improvement, two were discharged without symptomatic recurrence, and one was lost to follow-up. CONCLUSION: Airway stenting can be performed in the carina with high technical success using variable stent configurations. Although the rate of immediate symptomatic improvement is high, stent-related complications frequently occur.

Clinical manifestation and management of primary malignant tumors of the cervical trachea.

To explore clinical manifestation and therapies of primary malignant tumors of the cervical trachea, we retrospectively reviewed 31 patients with primary cervical tracheal malignant tumors diagnosed in the last 15 years by means of clinical manifestation, fiberoptic endoscopy, CT scanning and histopathological examinations. All of them were hospitalized and treated at the Second Xiangya Hospital, Central South University. Of them, 4 underwent emergent tracheotomy under local anesthesia, 9 were inserted with a laryngeal mask airway, 18 underwent tumorectomy under general anesthesia with endotracheal intubation, and of them 11 had tracheotomy during surgery. Of those 31 patients, tracheal malignant tumors in 9 cases were resected via laryngeal and retrograde tracheal incisions under endoscope; the tumors in 13 cases were excised via sleeve trachea resection and end-to-end anastomosis; those in 8 were removed by tracheofissure, and the tumor in 1 case was not excised surgically. Among the 30 resected patients, 20 patients received both radiotherapy and chemotherapy; 6 received radiotherapy only, and 4 did not receive any adjuvant therapies. During follow-up between 2 and 11 years, among 31 patients, there was no recurrence in 24 cases. Among the 7 deceased patients, 1 displayed multiple tracheal chondrosarcoma, 4 displayed adenoid cystic carcinoma, and 2 displayed squamous cell carcinoma. Emergency lower tracheotomy is necessary only when patients with tracheal, malignant tumors are in a critical condition. Sleeve trachea resection is the optimal therapy for tracheal malignant tumors. However, in the treatment of tracheal malignant tumors adjacent to the larynx or the involved trachea is over 6 cm in length, other surgeries shall be performed. Postoperative adjuvant radiotherapy and chemotherapy can achieve the same therapeutic effect as sleeve trachea resection.

Primary undifferentiated carcinoma of the trachea.

BACKGROUND: Primary undifferentiated or lymphoepithelial carcinoma mainly occurs in the nasopharynx. Tracheal localization is exceedingly rare. To the best of our knowledge, only four cases have been reported previously, all in Asian patients. CASE REPORT: A 61-year-old male European patient presented with hemoptysis and cough for several months. The workup revealed a primary tracheal tumor without regional or distant metastasis. The patient was treated with tracheal resection followed by end-to-end reconstruction. Pathologic analysis of the tumor, including immunohistochemistry, confirmed the diagnosis of lymphoepithelioma-like carcinoma. Testing for Epstein-Barr virus by hybridization in situ was massively positive. With a follow-up at 15 months, the patient is alive and free of disease. CONCLUSION: We report the first case of lymphoepithelioma-like carcinoma in the trachea in the European population. Treatment possibilities are discussed. They should be based on each patient's clinical presentation and the results of their preoperative workup.