Adenoid Cystic Carcinoma (Cylindroma)
Epidemiology
- Account for 40% of primary tracheal tumors
Physiology
- Malignant mucoepithelial tumor of lung
- Usually aggressive
Pathology
- Characteristic appearance of cylinders (on end view), resembles normal thyroid tissue
Diagnosis
- FOB: usually occurs as sessile/ polypoid mass in airway
Clinical
- Progressive Upper Airway/Tracheobronchial Obstruction (see [[Obstructive Lung Disease]])
- Clinical
- Dyspnea
- Chronic Hypoventilation (see Chronic Hypoventilation)
Treatment
- Bronchoscopic Removal: fulguration/laser may be used for poor surgical candidates
- Surgical Excision ( with wide margins): usually recommended
- Tumors may locally recur if not completely resected
- XRT: for positive surgical margins/ recurrent disease
- Used more commonly with salivary gland origin, less experience with airway origin tumors
- Chemo: palliative (for metastatic disease)
Prognosis
- May progress slowly over years
- May respond initially to XRT, but usually recurs if not initially resected
References