Revisit of Primary Malignant Neoplasms of the Trachea: Clinical Characteristics and Survival Analysis
Revisit of Primary Malignant Neoplasms of the Trachea: Clinical Characteristics and Survival Analysis Kuang-Yao Yang1, Yuh-Min Chen1, Min-Hsiung Huang2 and Reury-Perng Perng1
1Chest Department and 2Section of Thoracic Surgery, Veterans General Hospital-Taipei and School of Medicine, National Yang-Ming University, Taipei, Taiwan
A clinical review with an analysis of prognostic factors, including clinical characteristics, histological classifications, presenting symptoms/signs and treatment modalities, was conducted in 67 patients with primary malignant neoplasms of the trachea who were seen at the Veterans General Hospital-Taipei between 1979 and 1994. The incidence of tracheal cancer was 140 times less than lung cancer during this period. Delayed diagnosis of more than 6 months after onset of symptoms occurred in one-third of the patients owing to lack of specific symptoms/signs indicative of tracheal disease. Epidermoid carcinoma was the most frequent histological type encountered and accounted for half of the cases. Surgical resection was the first choice of treatment for all patients if the disease was locally confined, except for small cell carcinoma and lymphoma. Radiotherapy was given if the patient was not suitable for surgery. Single and multivariate analyses showed that clinical symptoms and signs were not related to prognosis, except for general malaise and acute respiratory failure. Patients suffering from adenoid cystic carcinoma and mucoepidermoid carcinoma had a better prognosis than other histological diagnoses. Whether the patient received radiotherapy or not proved to be a significant prognostic factor in the patients. Patients with tracheal cancers had a poorer prognosis than those with lung cancer.
Primary malignant neoplasms of the trachea are rare, accounting for 0.1% of all malignancies (1 ). Lung cancers were 180 times more frequently diagnosed than tracheal malignancies (2 ). The correct diagnosis is seldom made initially and a delay in diagnosis is common. This delay in diagnosis is most likely due to a lack of early symptoms or non-specific symptoms (1 -4 ). The rare reports in the literature not only show the rarity of tracheal carcinoma, but also indicate the limited experience with this malignancy at any given institution.
Detailed clinical manifestations and histological classifications were reported about two decades ago (1 -3 ). The treatment modalities for tracheal tumors have also been discussed but with no consensus (5 -11 ). Univariate survival analysis had been tried before (3 ,6 ). However, no well accepted statistical methods have been used. Also no multivariate analyses have been applied before. The present study was undertaken to investigate the clinical features, management and survival analysis of our patients using uni- and multivariate analyses.
From 1979 to 1994, there were 67 cases of histocytologically proven primary malignant neoplasms of the trachea in the Veterans General Hospital-Taipei, Taiwan. All tumors arising outside the trachea, including direct extension from adjacent organs or distant metastases to the trachea, were excluded. Primary bronchogenic carcinoma showing tracheal involvement by direct extension was also excluded. The clinical characteristics of the patients are shown in Table 1 . There were 55 men and 12 women. The mean age was 61 years with a range between 25 and 85 years. Forty-eight of 67 patients smoked. All patients received fiber-optic bronchoscopic examinations, chest radiography and CT scans. Whole-body bone scans and brain scans were performed if clinically indicated.
Surgical resection was the first choice of treatment if the disease was locally confined. Post-operative radiotherapy was optional. Radiotherapy was the primary treatment modality if the patient could not receive surgical treatment. Chemotherapy was the primary treatment modality in patients suffering from small cell carcinoma and malignant lymphoma. Chemotherapy was an optional treatment after initial treatment with surgery and/or radiotherapy. Drugs of choice were usually cyclophosphamide, epirubicin and cisplatin for cancers other than small cell carcinoma and lymphoma. Cisplatin and etoposide were given for patients with small cell carcinoma. Laser removal of the intratracheal tumor was the palliative treatment of choice. Mechanical ventilation was given to four patients who initially presented with acute respiratory failure. Patient survival time was calculated from the initial diagnosis of the primary disease to their death or last follow-up. Univariate survival analysis was based on the Kaplan-Meier estimate and log-rank test. Multivariate analysis was performed with the Cox regression method.
Epidermoid carcinoma was the most frequent histological type of malignant tracheal tumor found and accounted for 52.2% of cases, followed by adenocarcinoma (14.9%), poorly differentiated carcinoma (10.4%), adenoid cystic carcinoma (7.5%) and others (15%). The mean age of all patients was61 years. However, the mean age was only 29 and 41 years in mucoepidermoid carcinoma and adenoid cystic carcinoma, respectively. Adenocarcinoma and poorly differentiated carcinoma tended to occur in older patients. Delayed diagnosis of more than 6 months after symptoms onset occurred in one-third of patients. Median survival for all patients after diagnosis of malignant tracheal tumors was 6 months (Fig. 1 ). Median survival varied widely with different histological types of tracheal tumors. Those who suffered from adenoid cystic carcinoma or mucoepidermoid carcinoma had significantly better survival than other types of histology. Those who suffered from epidermoid carcinoma, adenocarcinoma or carcinosarcoma had worse survival than other types of cancer statistically. The median survival time was longest in the lymphoma group and shortest in the carcinosarcoma group (Table 1 ).
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