Cylindromes Thoraciques: Thoracic Adenoid Cystic Carcinomas

Adenoid cystic carcinomas in the trachea are rare, but represent around 40% of all tracheal tumours. Other intrathoracic localisations include the carena or proximal airways. Adenoid cystic carcinoma's growth rate is slow so that it is frequently diagnosed at an advanced stage. Pathological identification may be difficult. Treatment in limited tumours is based upon surgical resection often combined to radiotherapy because of close surgical margins. Radiotherapy dose may vary between 45 and 65 Gy according to margins status. Five-year survival rates of 65-80% have been reported after surgery or surgery and postoperative radiotherapy. Among inoperable patients treated with exclusive radiotherapy for tracheal tumours (including adenoid cystic but also squamous cell carcinomas of poorer prognosis), the recommended delivered dose should be over 60 Gy. Five-year survival rate in these very heterogeneous series may vary between 12 and 27%. Local or metastatic recurrences may occur very lately. They are considered chemo-resistant and targeted therapies may prove to be effective in the future.