Adenoid cystic carcinoma of the tracheobronchial tree

Adenoid cystic carcinomas of the tracheobronchial tree are a type of low-grade tracheal tumour. They are considered to be the second most common primary tumour of the trachea.

They are usually first recognized in patients in their 40s. There is no recognised gender predilection.

Symptoms in patients with adenoid cystic carcinoma are usually related to airway obstruction and therefore includes dyspnoea, cough, stridor, wheezing, and haemoptysis.

The tumors may be histologically classified into tubular, cribriform, and solid subtypes 6. The tumours are usually low grade but may occasionally undergo high grade transformation 7.

Risk factors

Smoking appears to affect the incidence.

Location

Adenoid cystic carcinomas usually arise in the lower trachea. Less commonly they are found in the mainstem bronchi, lobar bronchi and rarely, in the segmental bronchi and extrathoracic trachea.

CT

On chest CT, these tumours have a notable tendency toward submucosal extension and typically manifests as either:

  • intraluminal mass of soft-tissue attenuation with extension through the tracheal wall
    or
  • diffuse or circumferential wall thickening of the trachea, a soft-tissue mass filling the airway
    or
  • homogeneous mass encircling the trachea with wall thickening in the transverse and longitudinal planes

The longitudinal extent of the tumours are greater than their axial extent and the tumors usually involve more than 180° of the airway circumference.

They can be variable in shape ranging from polypoid to broad-based. Their margins are also variable and ranges from smooth to lobulated to irregular. Intratumoural calcification is rare.

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Article Information

rID: 24949
System: Chest
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Adenoid cystic carcinoma of tracheo bronchial tree
  • Cylindroma of the tracheo bronchial tree
  • Tracheobronchial adenoid cystic carcinoma
  • Tracheobronchial cylindroma
  • ACC of tracheobronchial tree
  • Adenoid cystic carcinoma of tracheo-bronchial tree
  • Adenoid cystic carcinoma of tracheobronchial tree

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