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 Table of Contents  
ORIGINAL ARTICLE
Year : 2021  |  Volume : 5  |  Issue : 3  |  Page : 111-115

Primary tracheal cancer: A regional cancer center experience


Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India

Date of Submission30-Aug-2021
Date of Decision13-Nov-2021
Date of Acceptance30-Nov-2021
Date of Web Publication14-Dec-2021

Correspondence Address:
Pooja Babbar
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru - 560 029, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/oji.oji_35_21

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  Abstract 


Background: Primary tracheal cancers (PTC) are uncommon, and the treatment methods vary considerably. Aim: The aim of the present study was to explore the clinical features, management, and prognosis of PTC in an Indian context. Methods: Nineteen patients of PTC were retrieved from the medical records over a period from January 2013 to December 2019. The clinical profile, histological features, and treatment details were recorded and outcomes are analyzed in terms of progression-free survival (PFS) and overall survival (OS). Results: Histological distribution for the cases were squamous cell carcinoma (SCC) (n = 12), adenoid cystic carcinoma (ACC) (n = 6) and small cell carcinoma (n = 1). All the patients were symptomatic. SCC was located more (7 out of 12 cases; 58.33%) in the lower third of the trachea than ACC (2 out of 6 cases; 33.33%). At initial diagnosis, five patients had metastatic disease and all the cases were of SCC histology (4 cases treated with palliative chemotherapy and 1 case received best supportive care). Among nonmetastatic cases (n = 14), 4 patients (SCC: 2; ACC: 2) were considered for primary surgery and the rest were considered unresectable and treated with other modalities except one case of ACC who did not come for treatment after diagnosis. The median PFS for ACC patients was higher than SCC (32 months vs. 10 months; P = 0.013). The median OS for ACC was higher than SCC cases (34.5 months vs. 11.2 months; P = 0.009). Conclusions: SCC followed by ACC are the most common histology types for PTC. ACC has a better prognosis compared to SCC.

Keywords: Adenoid cystic carcinoma, primary tracheal cancers, prognosis, squamous cell carcinoma


How to cite this article:
Babbar P, Rudresha A H, Lokanatha D, Arjunan R, Jacob L, Babu M C, Lokesh K N, Rajeev L K, Saldanha SC, Abhilash G H, Pandey A. Primary tracheal cancer: A regional cancer center experience. Oncol J India 2021;5:111-5

How to cite this URL:
Babbar P, Rudresha A H, Lokanatha D, Arjunan R, Jacob L, Babu M C, Lokesh K N, Rajeev L K, Saldanha SC, Abhilash G H, Pandey A. Primary tracheal cancer: A regional cancer center experience. Oncol J India [serial online] 2021 [cited 2022 Jan 21];5:111-5. Available from: https://www.ojionline.org/text.asp?2021/5/3/111/332510




  Introduction Top


Malignant primary tracheal tumors (PTT) are rare with a reported annual incidence of 1 in a million population and account for approximately 0.2% of respiratory tract malignancies and 0.02%–0.04% of all reported cancers.[1] The commonest histological subtypes are squamous cell carcinoma (SCC) followed by adenoid cystic carcinoma (ACC) and these two histology's account for 75% of all primary tracheal malignancies.[2]

Bronchoscopy and biopsy are commonly used to diagnose PTTs, but tumors are often discovered late in their course. Although complete surgical resection has been found to increase survival in maximum cases,[3] considering the disease's rarity and paucity of broad information, about the availability and efficacy of surgical options, most patients are under-treated and have a poor prognosis.[4] As a result, therapeutic strategies for malignant PTT patients have not been well-defined. During the previous decade, there is a significant development in radiotherapy, chemotherapy, and bronchoscopy intervention towards the treatment of primary tracheal cancers (PTCs), yet limited studies regarding the clinical outcomes have been stated. Malignant PTTs are a confounding type of cancers that cause major health problems for people. The scarcity of study on this group of patients is most likely owing to their rarity.[5] Therefore, the current study was carried out to determine the clinical features, management, and prognosis of malignant PTT.


  Materials and Methods Top


The present case series was conducted on diagnosed patients of malignant PTT who attended to a tertiary care cancer institute in Bangalore, India between January 2013 and December 2019. A total of 19 patients with malignant PTT were identified from the patients' records database. The trachea was characterized as extending superiorly from the cricoid cartilage to the carina inferiorly. All patients with lung or larynx cancer involving the trachea were excluded from the study. All patients underwent chest roentgenogram, computed tomography, and bronchoscopy evaluation after admission. In performing bronchoscopy, the biopsy was done simultaneously, and histological diagnosis was obtained. To identify the site of malignancies, the trachea was split into three equal sections: The upper, middle, and lower thirds. The tumor-related information was obtained from computed tomography scans and bronchoscopy reports.

The patients received different treatment modalities according to disease status. For nonmetastatic disease treatment modalities used are either upfront surgery ± adjuvant radiotherapy, or radical radiotherapy (RT)/concurrent chemoradiation (CCRT). Whereas, for metastatic diseases, patients were treated as palliative chemotherapy or palliative RT.

Statistical analysis

All analyses were conducted using the IBM SPSS Statistics for windows, Version 23.0. (Armonk, NY: IBM Corp.). Progression-free survival (PFS) was estimated using the Kaplan–Meier analysis. P < 0.05 was considered statistically significant.


  Results Top


The basic characteristics of the patient profile are depicted in [Table 1]. The median age of presentation was 54 years and there was a male preponderance with a male-to-female ratio of 1.7:1. According to histological diagnosis, PTT was distributed as SCC (n = 12), ACC (n = 6) and small cell carcinoma (n = 1). SCC was more likely to occur with increased age than ACC (median age: 58 years vs. 46 years). Tobacco addiction to smoking was found more in patients with SCC than ACC histology (75% vs. 50%). Anatomically, PTTs were distributed as 9 cases involved the lower third of the trachea, 6 cases involved the middle third and 4 cases involved the upper third of the trachea. SCC cases were located more in the lower third trachea (58.3%) whereas ACC cases more in the middle third trachea (50%) [Table 1]. ECOG PS is depicted in [Table 1].
Table 1: Basic characteristics of patient profile and according to major histological types

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All the patients were symptomatic, and the most common symptom was cough (84.2%), dyspnea (63.2%), hemoptysis (42.1%), hoarseness of voice (10.5%), and dysphagia (5.3%) of the patients. Tracheal strictures were found in 15 patients, and infiltration of tumor to the surrounding structure was present in 13 cases. At diagnosis, metastases to other organs were found in five patients, with the lung being the most common site of metastasis in all patients and bone lesion as an additional site of metastasis in one patient before the start of treatment and all cases were of SCC histology.

The mode of treatment for PPT in all patients is depicted in [Figure 1]. The treatment of PTT patients according to their histological types is depicted in [Table 2].
Figure 1: Mode of treatment

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Table 2: Therapeutic treatments for primary tracheal tumors according to the histological type

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Overall, only 4 patients were considered for primary surgery and rest were considered unresectable and treated with other modalities. Seven patients of SCC of trachea had nonmetastatic disease at diagnosis. Two patients of SCC with the tumor are located in the upper third of the trachea were treated by surgery only (tracheal sleeve resection plus anastomosis). No adjuvant treatment was given in view of early-stage disease, node-negative and margin negative resection in one patient and the other patient had noncompliance to adjuvant treatment. Rest five patients were treated with radical CCRT. In these CCRT schedule, 3 patients received weekly cisplatin based and rest 2 patients received weekly carboplatin-based concurrent chemotherapy. CCRT was given at a total RT dose of 60 Gy in 30 fraction at 2 Gy per fraction. Out of 5 metastatic SCC PPT patients, one case with lung metastasis and ECOG PS 3 planned for the best supportive care and died of rapidly progressive disease within 1 month of diagnosis. Rest four cases were treated in line of palliative chemotherapy with paclitaxel plus carboplatin-based combination regimen.

Out of 6 patients of ACC of the trachea, one patient did not come for treatment after diagnosis. Two patients underwent surgical resection due to localized disease. For these two cases, tracheostomy was performed first followed by laryngopharyngectomy and reconstruction as surgical procedures. Among these two, one patient received adjuvant radiotherapy with a total dose of 50 Gy/25 fractions in view of the presence of factors such as the extent of disease, close margins, and node-positive and the other patient had prolonged postoperative complication deferring adjuvant treatment. Two patients with locally advanced disease received RT with a total radiation dose of 60–64 Gy at 2 Gy per fraction. One patient with the presence of hemoptysis and extensive disease not amenable to definitive treatment received palliative radiotherapy with a total dose of 20 Gy at 4 Gy per fraction.

Patient of small-cell carcinoma of the trachea with ECOG PS 1 received radical CCRT as concurrent chemotherapy of cisplatin 50 mg/m2 on day 1 and day 8 plus etoposide 50 mg/m2 day 1–5 q four weekly for two cycles with radiation therapy to a total dose of 64 Gy at 2 Gy per fraction.

The median PFS (Kaplan–Meier) for patients with SCC was 10 months and for ACC it was 32 months as depicted in [Figure 2]. The difference of PFS between these 2 histological subtypes was found to be statistically significant with the P = 0.013. The median overall survival (OS) for PTC patients of ACC histology was significantly higher than that of SCC histology (34.5 months vs. 11.2 months; P = 0.009).
Figure 2: Progression-free survival between squamous cell carcinoma and adenoid cystic carcinoma

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  Discussion Top


In this study, we described the incidence pattern, clinical profiles, and outcomes of PTT patients in an Indian setting. Our retrospective analysis presented 19 patients diagnosed with PTT during the study period.

When one looks for the distribution of the histological types of tracheal cancer, most of the previous studies reported SCC as the most common type accounting for 43%–73%[6],[7],[8] of all tracheal tumors followed by ACC which represents around 7%–26%[1],[9] of cases. Few series even reported a higher percentage of ACC. Grillo and Mathisen[10] in a series of PTT reported 40% ACC, whereas Perelman et al.[11] in their series on PTT reported ACC as the predominant histology with more than 50% of total primary tracheal tumors. In our series, PTT with SCC histology was predominant in 63.2% of cases and ACC in 31.6% of cases.

In our study, patients with SCC were older (Median age 58) than those with ACC (Median age 46) which is similar to previous studies.[1],[8] Honings et al.[1] in a nationwide study on tracheal cancers in the Netherland reported the mean age of SCC at diagnosis (64.7 years) was higher than the of ACC (59.5 years). We found that PTT was commonly observed in males (63.16%) than females, this finding is in accordance with majority of studies.[5],[12],[13],[14] Majority of patients (68.4%) in our study are smoker which was supported by other published reports.[5],[12] Jiang et al.[5] in their study reported 70% of SCC patients as smoker and 40% of ACC patients as smoker. We also find that smoker are more seen in SCC (75%) than ACC (50%) patients.

Dyspnea, cough, hemoptysis, and shortness of breath are the common symptoms of presentation in tracheal tumors. However, few cases especially of ACC, and carcinoid histology may remain asymptomatic for months or years due to their slow-growing nature.[3] Majority of our patients were symptomatic and common symptoms were related to obstruction of the airway, i.e., cough (84%), dyspnea (63%), and hemoptysis (42%). Our data are supported by several published literatures.[5],[12] Licht et al.[12] reported productive cough (82%) and shortness of breath (75%) as the most common symptoms. Malignant PTTs are mostly occurred in the lower third of the trachea and showed worse survival outcomes, reason may be that tumors of lower anatomic location are more aggressive.[5],[15] Other studies are needed to confirm this finding. Jiang et al. stated that malignant PTTs are more reported in the lower third of the trachea than benign PTTs. We reported the lower third of the trachea (47.4%) as the most common location for malignant PTT. We found SCC cases more located at the lower third of the trachea than ACC cases (58.3% vs. 33.3%) which is supported by Jiang et al. (70% vs. 30%).[5]

The optimal treatment for PTTs remains controversial. Several studies suggest that extensive surgical resection with adjuvant RT or chemoradiation therapy should be the standard method of treatment. The percentage of patients where surgery is performed as first-line treatment widely varies among studies from 5% to 100%. In the current study, surgery was done in 4 patients out of 19 (21.05%), which is comparable with the study done by Napieralska et al.[16] The reason why surgery is considered in only a few cases may be that only few centers have gained experience in the management of tracheal malignancies because of their rarity.[13] Radiotherapy is indicated as an adjuvant after resection, tumors that are unresectable or medically inoperable, and for palliation of severe symptoms. In our series, radiotherapy was used in 10 patients for various indications such as radical CTRT (6 cases), radical RT (2 cases), adjuvant RT (1 case), and palliative RT (1 case). According to several studies, surgery was followed by adjuvant radiotherapy in 20%–100% of patients.[7],[16],[17] Chemotherapy was a part of the therapy in 0% to 29% of patients in large studies.[7],[12],[17] Cisplatin-based chemotherapy is the most commonly used drug in several studies.[16] In our series, chemotherapy was used in 10 patients which includes concurrent setting with radiation therapy in six patients with cisplatin as the most used chemotherapy, and palliative chemotherapy with paclitaxel and carboplatin regimen was given to four patients with lung metastasis.

Malignant PTTs of the lower third of the trachea have poor survival outcomes than the middle or upper third of the trachea. The possible reason for this may be due to predilection of SCC in the lower third of the trachea and the common hypothesis of tumors of lower anatomic location as more aggressive than upper location.[5] We also found poor survival in PTT cases located at the lower third of the trachea than located at the middle/upper third of the trachea. We found higher OS in upfront non-metastatic patients than upfront metastatic cases (33.2 months vs. 12.1 months; P = 0.0001). The median PFS for patients with SCC was 10 months and for ACC it was 32 months which is comparable with the published literatures.[18],[19] Honings et al. in a nationwide study in the Netherlands reported higher 1-year, 2-year, and 5-year survival for PTT patients of ACC histology than SCC histology.[1] Jiang et al. reported higher 3-year and 5-year survival in PTT patients with ACC histology than SCC histology although not statistically significant.[5]


  Conclusions Top


SCC followed by ACC are the most common PTTs. Surgical resection is the treatment of choice for PTT whenever possible and it improves prognosis, symptoms, and quality of life. Furthermore, radiotherapy is an important part of the treatment of patients with PTTs. In advanced unresectable malignant PTTs, radiation and bronchoscopy interventions such as stenting of the trachea serve as palliative therapy. The literature lacks studies examining chemotherapy in patients with tracheal cell cancers, and the role of chemotherapy remains unclear which requires further investigation. ACC has a better prognosis compared to SCC. Early diagnosis and definitive surgical therapy before local or distant spread of cancer is associated with favorable prognosis and increased survival. Additional studies are warranted to identify optimal screening and treatment options for this difficult disease.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Honings J, van Dijck JA, Verhagen AF, van der Heijden HF, Marres HA. Incidence and treatment of tracheal cancer: A nationwide study in the Netherlands. Ann Surg Oncol 2007;14:968-76.  Back to cited text no. 1
    
2.
Grillo HC. Development of tracheal surgery: A historical review. Part 2: Treatment of tracheal diseases. Ann Thorac Surg 2003;75:1039-47.  Back to cited text no. 2
    
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Nouraei SM, Middleton SE, Nouraei SA, Virk JS, George PJ, Hayward M, et al. Management and prognosis of primary tracheal cancer: A national analysis. Laryngoscope 2014;124:145-50.  Back to cited text no. 4
    
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Jiang M, Lei Q, Lv X, Zou L, Liu J, Meng J. Clinical features and prognosis analysis of 57 patients with primary tracheal tumors. Transl Cancer Res 2020;9:613-9.  Back to cited text no. 5
    
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Shao D, Gao Q, Cheng Y, Du DY, Wang SY, Wang SX. The prognostic value of 18F-fluorodeoxyglucos PET/CT in the initial assessment of primary tracheal malignant tumor: A retrospective study. Korean J Radiol 2021;22:425-34.  Back to cited text no. 6
    
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Urdaneta AI, Yu JB, Wilson LD. Population based cancer registry analysis of primary tracheal carcinoma. Am J Clin Oncol 2011;34:32-7.  Back to cited text no. 7
    
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Hajdu SI, Huvos AG, Goodner JT, Foote FW Jr., Beattie EJ Jr. Carcinoma of the trachea. Clinicopathologic study of 41 cases. Cancer 1970;25:1448-56.  Back to cited text no. 8
    
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Webb BD, Walsh GL, Roberts DB, Sturgis EM. Primary tracheal malignant neoplasms: The University of Texas MD Anderson Cancer Center experience. J Am Coll Surg 2006;202:237-46.  Back to cited text no. 9
    
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Grillo HC, Mathisen DJ. Primary tracheal tumors: Treatment and results. Ann Thorac Surg 1990;49:69-77.  Back to cited text no. 10
    
11.
Perelman MI, Koroleva N, Birjukov J, Goudovsky L. Primary tracheal tumors. Semin Thorac Cardiovasc Surg 1996;8:400-2.  Back to cited text no. 11
    
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Licht PB, Friis S, Pettersson G. Tracheal cancer in Denmark: A nationwide study. Eur J Cardiothorac Surg 2001;19:339-45.  Back to cited text no. 12
    
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Gaissert HA. Primary tracheal tumors. Chest Surg Clin N Am 2003;13:247-56.  Back to cited text no. 13
    
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Hazama K, Miyoshi S, Akashi A, Yasumitsu T, Maeda H, Nakamura K, et al. Clinicopathological investigation of 20 cases of primary tracheal cancer. Eur J Cardiothorac Surg 2003;23:1-5.  Back to cited text no. 14
    
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Zhao Y, Zhao H, Fan L, Shi J. Adenoid cystic carcinoma in the bronchus behaves more aggressively than its tracheal counterpart. Ann Thorac Surg 2013;96:1998-2004.  Back to cited text no. 15
    
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Napieralska A, Miszczyk L, Blamek S. Tracheal cancer – Treatment results, prognostic factors and incidence of other neoplasms. Radiol Oncol 2016;50:409-17.  Back to cited text no. 16
    
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Xie L, Fan M, Sheets NC, Chen RC, Jiang GL, Marks LB. The use of radiation therapy appears to improve outcome in patients with malignant primary tracheal tumors: A SEER-based analysis. Int J Radiat Oncol Biol Phys 2012;84:464-70.  Back to cited text no. 17
    
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Chen K, Yang Z, Zhang X, Zhao T, Zhang X, Li W, et al. Clinical features and prognosis of primary tracheal small cell carcinoma: A population-based analysis. Transl Cancer Res 2020;9:882-90.  Back to cited text no. 18
    
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