Adenoid Cystic Carcinoma
David Cassarino, MD, PhD
Key Facts
Terminology
Adenoid cystic carcinoma (ACC)
Rare adnexal tumor showing prominent cribriform pattern and perineural invasion
Clinical Issues
Up to 70% of cases reported to recur (due to perineural invasion)
Only a few cases have metastasized, usually to lymph nodes and lungs
Microscopic Pathology
Composed of lobules, islands, and cords of basaloid cells
Numerous cystic and ductular spaces with prominent cribriform pattern
Cells are typically small, relatively monomorphic-appearing, and cuboidal-shaped
By immunohistochemistry, ACC stains positive with EMA, CEA-M, cytokeratins, p63, S100, and actin
 Adenoid cystic carcinoma at low magnification shows a dermal-based basaloid neoplasm with numerous cribriform spaces  containing basophilic mucin and showing a prominent Swiss cheese-like pattern. |
 Adenoid cystic carcinoma frequently shows perineural invasion, which accounts for its high local recurrence rate. Here, a large central nerve  is surrounded by multiple tumor cords and ducts. |
TERMINOLOGY
Abbreviations
Adenoid cystic carcinoma (ACC)
Synonyms
Primary cutaneous adenoid cystic carcinoma
Definitions
Rare adnexal tumor showing prominent cribriform pattern and perineural invasion
ETIOLOGY/PATHOGENESIS
Unknown
May be related to chronic sun damage in some cases
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