Adenoid Cystic Carcinoma (Cylindroma)

(aka 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
    • EBB: diagnostic

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

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