Adenoid cystic carcinoma of the lung

Definition

A malignant epithelial neoplasm, the counterpart if that occurring in the salivary gland.

Epidemiology

Adenoid cystic carcinoma constitutes less than 1% of all carcinomas of the lung.

Clinical features

90% of cases occur in the trachea, main stem or lobular bronchi5,7, where they cause shortness of breath, cough, wheeze or haemoptysis.

Radiology

There is a centrally located mass which may extend into lung parenchyma or into mediastinal fat.

Macroscopic appearances

Polypoidal lesions or plaques result in thickening of the bronchial submucosa. The infiltrative margins extend well beyond the obvious mass. Staging is by the TNM system.

Histopathology

Epithelial cells grow in a characteristic cribriform, solid, tubular or glandular pattern4,5 around a basement membrane rich extracellular matrix. The matrix may be hyalinised or mucinous. The tumour cells are small with little cytoplasm and darkly hyperchromatic nuclei. Mitotic figures are infrequent. Where tubules are multilayered, the luminal cells are low cuboidal and the outer cells form a myoepithelial layer. 40% of cases show perineural invasion. The tumour often extends beyond its macroscopically apparent limits3.

Immunohistochemistry

	Cytokeratins	positive
	Vimentin	positive
	SMA	positive
	Calponin	positive
	S-100	positive
	p63	positive
	GFAP	positive
	Collagen IV	basement membrane is positive
	Laminin	basement membrane is positive

Differential diagnosis

Management

Resection1 plus radiotherapy3. Radiotherapy alone may give prolonged control3.

Prognosis

Behaviour is usually indolent, with multiple local recurrences preceding late metastases7. The mean survival is ten years3.

References

Tumours of the Lung, Pleura, Thymus and Heart. WHO Classification of Tumours. IARC Press 2004.

1 Conlan AA, Payne WS, Woolner LB, et al. Adenoid cystic carcinoma (cylindroma) and mucoepidermoid carcinoma of the bronchus. Factors affecting survival. J Thorac Cardiovasc Surg 1978; 76:369-77.

2 Hills EA Cylindroma of the trachea and left main bronchus. Proc R Soc Med 1971; 64:221-2

3 Maziak DE, Todd TR, Keshavjee SH, et al. Adenoid cystic carcinoma of the airway: thirty-two-year experience. J Thorac Cardiovasc Surg 1996; 112:1522-31; discussion 1531-2

4 Moran CA, Suster S,Koss MN Primary adenoid cystic carcinoma of the lung. A clinicopathologic and immunohistochemical study of 16 cases. Cancer 1994; 73:1390-7

5 Nomori H, Kaseda S, Kobayashi K, et al. Adenoid cystic carcinoma of the trachea and main-stem bronchus. A clinical, histopathologic, and immunohistochemical study. J Thorac Cardiovasc Surg 1988; 96:271-7

6 Payne WS, Ellis FH, Jr., Woolner LB, et al. The surgical treatment of cylindroma (adenoid cystic carcinoma) and muco-epidermoid tumors of the bronchus. J Thorac Cardiovasc Surg 1959; 38:709-26

7 Prommegger R,Salzer GM Long-term results of surgery for adenoid cystic carcinoma of the trachea and bronchi. Eur J Surg Oncol 1998; 24:440-4

This page last revised 29.3.2005.