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Wednesday, May 17, 2017

Adenoid Cystic Carcinoma: Malignant Tumours of Salivary Glands

Other names:
1. Cylindroma
2. Adenocystic Basal Cell Carcinoma (BCC)
3. Pseudo-Adenomatous BCC
4. Basaloid mixed tumours

Definition:
It is a slow growing but aggressive neoplasm (recurrence is frequent), characterized by proliferation of ductal (luminal) and myoepithelial cells in cribriform, tubular, solid or cystic patterns.

Clinical features:
Same as mucoepidermoid carcinoma or pleomorphic adenoma except:
- Early local pain
- Fixation to deeper tissues
- Local invasion to perineural space and nerves

Histologic features:
1. composed of variable ductal cells and myoepithelial cells

2. Three growth patterns:
   a) Cribriform pattern: 
   - Classical, most common and best recognized pattern
   - Contains both luminal and non-luminal cells i.e. shows basaloid epithelial cell nests that form multiple cylindrical cyst like pattern called 'Swiss cheese' or 'honey comb' pattern

b) Tubular pattern: 
   - Shows only luminal/ ductal cells with single layer of basaloid cells or myoepithelial cells

c) Solid pattern:
- Shows non-luminal cells i.e. Solid groups of cuboidal cells with little tendency towards duct or cyst formation

Treatment: Surgical excision

Prognosis: Poor

MCQS


1. 'Swiss cheese' or 'honey comb' pattern is characteristic feature of:
   a. Pleomorphic adenoma
   b. Adenoid cystic carcinoma
   c.Mucoepidermoid carcinoma
   d. Acinic cell carcinoma
Correct answer is b.

2. Cylindroma is another name of
   a. Pleomorphic adenoma
   b. Warthin's tumour
   c.Mucoepidermoid carcinoma
   d. Adenoid cystic carcinoma
Correct answer is d.

3. Pseudo-Adenomatous BCC is
   a. Pleomorphic adenoma
   b. Warthin's tumour
   c.Mucoepidermoid carcinoma
   d. Adenoid cystic carcinoma
Correct answer is d.

4. Cribriform, tubular, solid or cystic patterns of myoepithelial cells is a feature of:
   a. Pleomorphic adenoma
   b. Warthin's tumour
   c. Adenoid cystic carcinoma
   d. Mucoepidermoid carcinoma
Correct answer is c.

5. Perineural space invasion is a characteristic feature of
   a. Pleomorphic adenoma
   b. Warthin's tumour
   c. Adenoid cystic carcinoma
   d. Mucoepidermoid carcinoma
Correct answer is c.

6. Classical or most common pattern of Adenoid cystic carcinoma is
   a. Cribriform
   b. Solid
   c. Tubular
   d. None of the above
Correct answer is a.