Twenty two year survival after incomplete resection of advanced adenoid cystic bronchogenic carcinoma

Abstract

In 1972, a 42 year old patient underwent thoracotomy for adenoid cystic carcinoma of the left main-stem bronchus. Pneumonectomy was performed, but the resection was not complete as the tumour had invaded the aortic and tracheal wall. The patient did not receive postoperative radiotherapy.In 1993, the patient was readmitted with severe respiratory insufficiency due to complete endobronchial tumour obstruction of the intermediate bronchus and extensive tumour growth in the mediastinum. Endobronchial laser treatment followed by stent implantation led to immediate symptomatic relief. The tumour then responded well to combined endobronchial and percutaneous radiotherapy. The patient died 7 months after readmission from ileus due to pancreatitis.We conclude that patients with advanced adenoid cystic carcinoma may profit from palliative surgery with respect not only to symptoms but also to duration of life. Eur Respir J., 1996Respir J., , 9, 1560 Adenoid cystic carcinoma of the bronchial system is a rare malignancy usually located in the central airways, i.e. the trachea or main-stem bronchi [1]. It appears to be unrelated to smoking, with an equal distribution in males and females. The tumour most often infiltrates the adjacent structures in continuity. Metastases to regional lymph nodes or distant sites are less common. Long-term survival has been reported for patients when the tumour has been resected [2,3], but local recurrences of tracheal tumours have been recorded even after 25 and 30 yrs [4]. Case reportA 42 year old male nonsmoker was admitted in December 1971 with almost complete atelectasis of the left lung. Bronchoscopy revealed an obstructing tumour in the proximal left main-stem bronchus. Thoracotomy showed that the tumour had grossly invaded peribronchial structures, including the aortic wall and the tracheal wall. Numerous enlarged lymph nodes were found. Pneumonectomy was performed, but residual tumour remained in the aortic wall and the bronchial surgical margins.Histologically, adenoid cystic carcinoma was diagnosed. The tumour showed trabecular and tubular structures filled with alcian blue-and periodic-acid-Schiff (PAS)-positive mucin. The surgical margins were positive, but none of the enlarged lymph nodes were involved by the tumour (tumour, node, metastasis classification (TNM) stage pT4N0M0 [5]). As an additional finding, epi-thelioid cell granulomatas with necrosis were detected within the resected lung, suggesting pulmonary tuberculosis. Antituberculosis treatment with isoniazid and ethambutol was given for 18 months; sputum cultures remained negative. The patient did not receive postoperative radiotherapy.In May 1993, the patient was readmitted with a 2 week history of severe respiratory insufficiency at rest. Bronchoscopy revealed complete tumour obstruction of the whole length of the intermediate bronchus, and extrabronchial compression of the right main-stem bronchus. Histologically, the tumour was again identified as adenoid cystic carcinoma of tubular subtype ( fig. 1). Compu...