Primary tracheal malignant neoplasms: the University of Texas MD Anderson Cancer Center experience

BACKGROUND: Primary malignant neoplasms of the trachea are very rare and data relating to them are limited. This study was conducted to review the presentation, management, and outcomes of primary tracheal cancers at our institution, a large multidisciplinary cancer center. STUDY DESIGN: Retrospective chart review was conducted for all patients found to have a pathologic diagnosis of primary tracheal malignancy. RESULTS: Since 1945, 74 patients were diagnosed with primary tracheal cancers. Among these, 34 (45.9%) were squamous cell carcinomas, 19 (25.7%) were adenoid cystic carcinomas, and 21 (28.4%) were of other histologic types. Presenting symptoms were most frequently dyspnea (55.4%), hemoptysis (48.6%), cough (41.9%), and hoarseness (35.1%). Most patients (77.3%) were former or current smokers, particularly those with squamous cell carcinoma (93.3%). For the entire group of 74 patients, the 5-year disease-specific mortality rate was 72.9% and the 5-year all-cause mortality rate was 79.3%. Patients who had adenoid cystic carcinoma and those with cervical primaries had better rates of disease-specific and overall survival than others (p = 0.036 and 0.006 for the former patient group and p = 0.006 and 0.030 for the latter patient group). Among patients with incident disease treated at our institution (n = 45), those undergoing primary operation with adjuvant radiotherapy appeared to have better disease-specific and overall survival rates compared with those undergoing primary radiotherapy with or without chemotherapy (p = 0.0002 and 0.0003, respectively). Although those undergoing operation and receiving radiotherapy did better than those undergoing operation alone, the difference was not statistically significant. CONCLUSIONS: Primary tracheal cancers are very rare, and our results should be viewed with caution, given that our population comprised a small heterogeneous group treated over a 60-year period. Although squamous cell carcinoma was the most common pathology in smokers, adenoid cystic carcinoma was more prevalent among nonsmokers. Operation with adjuvant postoperative radiotherapy is recommended for most patients.     

Primary tracheal tumors: experience with 14 resected patients.

OBJECTIVE: Primary tracheal tumors are rare. Management includes interventional endoscopy, surgery and radiotherapy. METHODS: Between 1987 and 1996, 14 patients treated by resection and reconstruction of the trachea and bifurcation for primary tracheal tumors were retrospectively analyzed. RESULTS: The most common histological finding was adenoid cystic carcinoma (n=7), followed by a squamous cell carcinoma (n=2), a mucoepidermoid carcinoma (n=2), a carcinoid tumor (n=1) and two benign tumors (xanthogranuloma, pleomorphic adenoma). Various reconstruction techniques were used and one prosthesis was implanted. Eight of the patients required preoperative Nd-YAG laser recanalisation. Six were treated by postoperative external beam radiotherapy, in three cases combined with endoluminal brachytherapy. Two major postoperative wound-healing impairment at the anastomosis occurred. Four minor wound-healing disorders were successfully treated by interventional endoscopy. Two patients died postoperatively with mediastinitis respectively with bilateral pneumonia. A local recurrence was observed in only two cases. At the last follow-up in January 1998, nine patients were still alive. We observed five long-term survivors (>6 years) with an adenoid cystic carcinoma or mucoepidermoid carcinoma. CONCLUSIONS: Extensive segmental resection of the trachea is the treatment of choice for primary malignant and occasionally for benign tracheal tumors. Interventional endoscopy is a part of modern tracheal surgery.     

Contemporary staging and prognosis for primary tracheal malignancies: a population-based analysis.

OBJECTIVE: Determine staging characteristics and survival outcomes for primary malignancies of the trachea. Design Cross-sectional analysis of national cancer database. METHODS: Cases of primary tracheal malignancy were extracted from the Surveillance, Epidemiology, and End Results database for the time period 1988-2000. T-stage, N-stage, and overall stage of presentation were determined. Mean, median, and 5-year survival statistics were computed using Kaplan-Meier survival analysis for each tumor histology and for the overall cohort according to stage. RESULTS: Ninety-two cases with adequate histologic information were identified. Mean age at presentation was 59.3 years with an equal sex distribution. Squamous cell carcinoma was the most common tumor type (41 cases) followed by adenoid cystic carcinoma (19 cases). Forty-nine cases (53%) presented with stage 3 or stage 4 disease. Squamous cell carcinoma exhibited poorer survival (mean survival, 44.0 month, 5-year survival, 34%) than adenoid cystic carcinoma (mean survival, 115 month, 5-year survival, 78%). Five-year unadjusted survival rates according to overall stage were 52.8%, 70.0%, 75.0%, 15.1%, respectively. CONCLUSIONS: Primary tracheal malignancies often present with advanced stage. Patients with squamous cell carcinoma of the trachea have poorer prognoses when compared with adenoid cystic carcinoma and other tumor types. Staging tracheal cancer with a TNM-based system helps predict survival. EBM rating: C.     

Surgical treatment of patients with stenosis of the central airway due to tracheal tumours

This study retrospectively evaluated bronchoscopic and surgical treatments for patients with central airway stenosis due to tracheal tumours. Seven patients treated by resection and reconstruction of the trachea for tracheal tumours between 1994 and 2008 were retrospectively reviewed. The most common histological finding was thyroid carcinoma (n = 3), followed by adenoid cystic carcinoma (n = 2), a metastatic thyroid tumour (n = 1), and a benign granular cell tumour (n = 1). Three of the patients required preoperative laser treatment (Nd:YAG) for recanalization. Five patients underwent end-to-end anastomosis for reconstruction. There was no postoperative mortality or morbidity such as anastomotic insufficiency of the reconstructed trachea. Three patients with a microscopic residual tumour required postoperative external radiotherapy. Surgical resection of malignant tracheal tumours is recommended not only for curative purposes, but also for reduction of the risk of smothering.     

Primary tracheal tumors: treatment and results

One hundred ninety-eight patients with primary tracheal tumors were evaluated in 26 years. One hundred forty-seven tumors were excised (74%): 132 (66%) by resection and primary reconstruction, seven by laryngotracheal resection or cervicomediastinal exenteration, and eight by staged procedures. Eleven more were explored. Forty-four squamous cell carcinomas were resected, 60 adenoid cystic, and 43 assorted tumors, benign and malignant. Eighty-two patients underwent tracheal resection with primary reconstruction, and 50 had carinal resection and reconstruction. Surgical mortality for resection with primary reconstruction was 5%, with one death after tracheal and six after carinal repair. Six patients had stenosis after tracheal or carinal resection; all underwent reresection successfully. Nearly all patients with squamous or adenoid cystic carcinoma were irradiated postoperatively. Twenty of 41 survivors of resection of squamous cell carcinoma are living free of disease (some for more than 25 years), 39 of 52 with adenoid cystic carcinoma (up to nearly 19 years), and 35 of 42 with other lesions (5 lost to follow-up). Comparison of length of survival of patients with squamous cell carcinoma and adenoid cystic carcinoma who are alive without disease with those who died with carcinoma supports surgical treatment (usually followed by irradiation). Positive lymph nodes or invasive disease at resection margins appear to have an adverse effect on cure of squamous cell carcinoma; such an effect is not demonstrable with adenoid cystic carcinoma.     

Tracheal tumors: surgical management.

In a 15-year period, 63 patients with primary tracheal tumors were seen. Twenty-eight patients with primary tumors and 8 with secondary tumors of the trachea were treated by resection with single-stage reconstruction. There were 24 cylindrical resections of trachea, 2 lateral resections of trachea, and 10 carinal reconstructions. Thirty-five additional patients with primary tracheal tumors were managed by staged reconstruction, irradiation, or no treatment. The most common primary lesion was squamous cell carcinoma and the second, adenoid cystic carcinoma. Benign primary tumors and low-grade malignant tumors obtained excellent palliation and usually cure. Surgical removal of squamous cell carcinoma and adenoid cystic carcinoma, usually with adjunctive irradiation, provided good palliation or the probability of cure. Resection of selected secondary tumors provided long-term palliation.     

气管及其隆突部肿瘤的外科治疗与预后

目的 总结分析气管及其隆突部肿瘤的临床表现、诊断、手术方法以及预后.方法 回顾性分析1986年6月至2005年6月手术治疗的32例气管及其隆突部肿瘤患者的临床资料,其中男性22例,女性10例,年龄14～63岁,中位年龄48岁.32例患者中气管肿瘤切除+端端吻合10例;全肺隆突切除+气管与主支气管端端吻合8例(右侧6例,左侧2例);右上肺隆突袖式切除重建术4例;隆突切除重建术4例;气管开窗行肿瘤及气管壁部分切除6例,其中2例因气管壁切除范围过大,以涤纶布内衬修补.结果 32例中鳞状细胞癌19例,腺样囊腺癌8例,腺癌2例,类癌1例,平滑肌肉瘤1例,腺瘤1例.手术并发症包括术后1例胸腔感染,3例出现心律失常.全组患者无手术死亡.随访时间5个月～3年,随访率100%.Kaplan-Meier法计算1、2和3年生存率为93.7%、59.4%和50.0%.结论 鳞状细胞癌和腺样囊性癌是气管及其隆突部肿瘤最常见的组织类型,术前气管镜和CT可帮助诊断,手术方式的正确选择是提高治疗效果的关键.     

原发性气管癌44例临床分析

比较原发性气管癌手术与非手术治疗的疗效，探讨影响本病预后的因素。回顾性分析经组织学证实的４４例原发性气管癌病人的临床与随访资料。手术切除１８例；非手术治疗２６例（放疗采用钴６０或高能Ｘ线，靶区剂量为６０～７０Ｇｙ；化疗采用ＣＯＭＦ或ＡＭＦＰ方案４～６个疗程）。结果采用ＫａｐｌａｎＭｅｉｅｒ法计算其１、３、５年生存率，手术切除者分别为８３．３％、６６．７％和３３．３％，非手术治疗者分别为７３．１％、４２．３％和１５．４％。ｌｏｇｒａｎｋ检验显示无统计学意义，Ｐ＞０．０５。气管上段腺样囊性癌预后相对较好，治疗后５年内约有７７．３％病人死于肿瘤末控、复发或转移。结论：原发性气管癌手术与非手术者的预后似乎无明显差异，影响本病预后的因素主要为其发病部位和病理类型     

Surgical treatment of tracheal and carinal tumors

Tracheal tumors are rare. The two most common histologic types (occurring in about equal numbers) are adenoid cystic carcinoma and squamous cell carcinoma. Current approaches for tracheal mobilization allow safe resection of up to 50% of the tracheal length and primary anastomosis in the vast majority of patients.     

Management of tracheal tumors

Since 1962, 110 primary tracheal tumors have been seen including 43 squamous cell carcinomas, 38 adenoid cystic, and 29 varied. Sixty-nine patients underwent resection of their primary tumors; an additional 33 patients with secondary tumors involving the trachea also underwent resection. Seventy-three of these 102 patients underwent primary reanastomosis. In 17, laryngotracheal resection was required; staged resections were done for the rest. Survival data indicate that benign tumors are cured by resection with reconstruction and that patients with squamous cell carcinoma, adenoid cystic carcinoma, and other types of malignant lesions obtained either long-term palliation or cure if surgical resection was possible. In patients with selected types of secondary tumors, resection and reconstruction provide prolonged palliation.     

Tracheal cancer in Denmark: a nationwide study.

OBJECTIVE: Most published series on tracheal cancer reflect single institution experiences. We used the nationwide Danish Cancer Registry to report on characteristics and treatment of tracheal cancers in Denmark. METHODS: One hundred and nine cases of primary tracheal cancers were extracted from the registry in the period 1978-1995. The clinical data, histological distribution and treatment modalities were analyzed. The cancers were staged in four groups (stage I-IV) according to size, location and spread. RESULTS: Seventeen cases were diagnosed at autopsy. Ninety-two cases were diagnosed in vivo and 84% of these within 3 months after the first consultation. Sixty-three percent of the cancers were squamous cell carcinomas and only 7% were adenoid cystic carcinomas. The disease was at stage I in 21%, stage II in 23%, stage III in 6% and stage IV in 50%. The majority of the patients received radiotherapy as single treatment. Only nine patients were offered surgery (six were resected and three were found inoperable). The overall survival rates for cases diagnosed in vivo were 1-year 32%, 2-year 20% and 5-year 13%. For the resected patients the 5- and 15-year survival rates were 50%. CONCLUSIONS: Tracheal cancers were rare and adenoid cystic carcinomas not as frequent as generally believed. Surgery was rarely offered. A resectability rate of only 10% is not adequately explained by selection bias and indicates a nihilistic attitude based on ignorance about surgical treatment of tracheal cancers. A more dedicated and aggressive approach with centralized workup and radical treatment is strongly recommended.     

A case of adenoid cystic carcinoma of the trachea resected surgically after Nd-YAG laser therapy

A case of primary tracheal tumor ablated by endoscopic Nd-YAG laser prior to tracheal sleeve resection was reported. A 53-year-old man had dry cough, bloody sputum and dyspnea. Bronchoscopic examination revealed polypoid tumor which located 2 cm below the vocal cords and obstructed the airway almost completely. After tumor vaporized by Nd-YAG laser, bronchoscopy type 200 could pass through the tracheal lumen and the length of the lesion was diagnosed within 4 cm. Biopsy specimen was confirmed adenoid cystic carcinoma. Cervical trachea was resected of six tracheal cartilage (first to sixth cartilage) combined with left thyroid lobe under collar incision and median sternotomy. The neck was fixed for ten days by tying the jaw to the anterior chest wall in order to reduce excessive tension to the anastomosis. Pathological examination revealed the tumor residual microscopically, 60 Gy of radiotherapy was conducted.     

Tracheobronchial tumors: an eighteen-year series from Capital Hospital, Peking, China

From 1961 to 1979 in Capital Hospital, Peking, China, 27 patients with tracheobronchial tumors were treated: 20 underwent operation and 7 were managed by palliative measures. All 27 patients had an intraluminal lesion of the trachea or major bronchi. Respiratory obstruction was the main clinical manifestation of the tracheal tumors. Chronic suppurative infection of the lung was the principal clinical manifestation of the bronchial tumors. Histological diagnosis of these 27 patients revealed ten different cell types: squamous cell carcinoma (10 patients, including the 7 who did not undergo operation); adenoid cystic carcinoma (5 patients); carcinoid, hamartoma, neurofibroma, and papilloma (2 patients each, respectively); and leiomyoma, hemangioma, chondroma, and teratoma (1 each, respectively). The operative methods used in 20 patients were lateral resection of tracheal wall (2 patients), resection of left main bronchial root (1), local excision (5), lobectomy (5), pneumonectomy (3), bronchoscopy (1), and exploratory operation and tracheostomy (3). Local excision or lateral wall resection of the trachea was chosen for benign or less dangerously malignant tracheal tumors. Lobectomy or pneumonectomy was performed for the secondarily infected, destroyed lungs in patients with bronchial tumors.     

Acute thoracic inlet obstruction in achalasia with adenoid cystic and squamous cell carcinoma

Respiratory symptoms due to compression of the trachea by the dilated esophagus in achalasia are extremely rare. A patient is presented whose respiratory manifestations included engorged neck veins and a neck swelling that fluctuated with respiration. He also had two malignant tumors in his dilated esophagus, a squamous cell carcinoma and an adenoid cystic carcinoma.     

Upper Lobectomy for Lung Cancer with True Tracheal Bronchus: A Unique Presentation

Tracheal bronchus is an aberrant bronchus usually originating from the right lateral wall of the trachea, with an incidence ranging from 0.1% to 5% and usually within 2.0cm above the carina. The incidence of lung cancer with bronchial anomaly is very rare. Only nine cases of lung cancer developing from the tracheal bronchus have been reported in literature. Histological examination showed squamous cell carcinoma in only three of them, and we present a fourth case, in a 57-year-old man. Interestingly, our patient's anomaly included both an absence of the right upper bronchus and the fact that the right upper lobe was ventilated by the true tracheal bronchus. This is the first documented case in the world of a squamous carcinoma originating in the true tracheal bronchus. Post-surgical histological stage was T2aN0M0 (stage IB). The patient is in a good condition 48 months after the operation and has no evidence of recurrence.     

Treatment outcome of resected and nonresected primary adenoid cystic carcinoma of the lung.

BACKGROUND AND OBJECTIVE: The incidence of primary adenoid cystic carcinoma of the lung is relatively rare and the optimal treatment strategy is still unclear. METHODS: Sixteen adenoid cystic carcinoma patients were treated at our institute from 1972 to 1998 and their clinical features, treatments and survivals were reviewed. RESULTS: Half of all patients were female and the median age was 46 ranging from 30 to 64. All primary lesions were located in the central bronchial tree and 80% of the patients had some symptoms. Eleven patients underwent a resection of the tumor with/without plasty of the trachea or bronchus. Although 6 (55%) of 11 patients had a microscopic residual tumor after resection, 5 patients who received postoperative radiotherapy survived without recurrence from 3 to 17 years. Five patients received radiotherapy as their initial treatment and all tumors responded well to the treatment. The 5-year and 10-year survival rates were 91 and 76% without local recurrence in the resected group and 40 and 0% in the nonresected group, respectively. CONCLUSION: These observations suggest that surgical resection should be selected first whenever possible, and, in addition, adenoid cystic carcinoma is sensitive to radiotherapy.     

Incidence and Treatment of Tracheal Cancer: A Nationwide Study in The Netherlands

Background The aim of this study was to assess the incidence, characteristics, treatment, and survival of patients with tracheal malignancies in the Netherlands. Methods All cases of tracheal cancer entered into the database of the Netherlands Cancer Registry in the period 1989–2002 were selected. Data on histological type, age at time of diagnosis, treatment, and survival were analyzed retrospectively. Results The annual incidence was 0.142 per 100,000 inhabitants (308 cases, of which 15 were found incidentally at autopsy). Of these, 72% were men. In 52.9%, the histological type was squamous cell carcinoma and in only 7.1% adenoid cystic carcinoma (ACC). Mean age at time of diagnosis was 64.3 years. Of the 293 patients diagnosed while alive, 34 patients underwent surgical resection (11.6%), 156 patients received radiotherapy (53.2%), and 103 patients neither (35.4%). Median survival of all 293 patients was 10 months (mean 28 months) with 1-year, 5-year, and 10-year survival rates of 43%, 15%, and 6%, respectively. The prognosis of patients with ACC was significantly better. The 5-year survival rate in patients who underwent surgical resection was 51%, and the 10-year survival rate in these patients was 33%. Conclusion The prognosis of patients with a tracheal malignancy is usually poor. Surgical treatment, however, can lead to good survival rates; still, this is currently only used in selected patients, even though it would seem to be possible in more cases in view of the technical advances in the field of tracheal surgery. Centralizing the care and treatment of tracheal cancers and implementing a more assertive attitude towards this disease could make surgery accessible to a larger number of patients. Data from the literature show that this would lead to better survival in patients with a tracheal malignancy.     

Malignant tumor of the trachea in children: diagnostic pitfalls and surgical management

Primary tracheal malignant neoplasms are very rare. Histologically, squamous cell and adenoid cystic carcinomas are the most common types of malignant primary tracheal tumors when all age groups are studied. In the past 5 years, we treated 2 children with tracheal mucoepidermoid carcinoma. Herein we report both cases and review the literature on the subject with particular emphasis on diagnosis and surgical management.     

Clinicopathological investigation of 20 cases of primary tracheal cancer.

OBJECTIVE: Primary tracheal cancer is considered to be relatively rare. Its epidemiology, therapeutic strategy and prognosis are not well understood. METHODS: We retrospectively investigated the clinicopathological aspects of 20 patients with primary tracheal cancer. RESULTS: Patients included 11 men and nine women with a mean age of 57.3 years. There were 12 squamous cell carcinomas and eight adenoid cystic carcinomas. Four patients received only palliative therapy. Sixteen patients underwent surgical treatment such as segmental tracheal, laryngotracheal, or carinal resection. One patient with squamous cell carcinoma died of postoperative mediastinitis. Although resected specimens from five patients had tumor positive margins, only one of those patients died after local recurrence and only three patients had postoperative treatment. The 5-year survival rate for patients who underwent surgery was 72.3%. CONCLUSIONS: Surgical treatment is the first choice therapeutic modality for primary tracheal cancer in consideration of its prognosis. While performing the operation, safety of the anastomosis should take precedence over completeness of resection.     

氩等离子体凝固术治疗气管腺样囊性癌19例效果分析

目的评价经支气管镜氩等离子体凝固术(argon plasma coagulation, APC)在气管支气管腺样囊性癌治疗中的临床应用价值。 方法回顾性分析2000年1月至2017年12月解放军总医院呼吸科收治的原发性气管支气管腺样囊性癌患者19例,其中12例气管腺样囊性癌、7例支气管腺样囊性癌,均行经支气管镜氩等离子体凝固术治疗。 结果19例患者进行了6～132个月(平均58.11个月)的随访,定期行胸部CT和支气管镜检查,治疗后近期有效率为100%,咳嗽、胸闷、气短等症状明显改善。其中完全有效10例(53%)、部分有效5例(26%)、轻度有效4例(21%)。19例患者气促指数,治疗前(3.5± 0.5)级、治疗后(1.5±0.6)级,差异有统计学意义(t＝6.2,P<0.05)。无严重并发症。 结论APC对于气道腔内病变的治疗安全有效,可有效切除肿瘤,保证气道通畅,迅速改善临床症状,为气管支气管腺样囊性癌提供一种新的治疗选择。