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Basal Cell Adenocarcinoma |
Adenoid Cystic Carcinoma |
Age |
Adults, with peak in sixth and seventh decades |
Typically adults, peak incidence in sixth decade |
Location |
Usually parotid gland, less commonly submandibular gland or minor salivary glands |
Parotid gland is most common (40%-50%) but may arise in any major or minor salivary gland |
Symptoms |
Painless, slow-growing mass. Some (especially membranous subtype) may be multinodular. Patients may also have skin adnexal tumors |
Slowly growing swelling, sometimes pain or paralysis due to perineural invasion. Other site-specific symptoms in minor salivary locations (e.g., nasal obstruction or epistaxis for sinonasal tumors) |
Signs |
Variably circumscribed, solid nodules |
Palpable nodules that may become fixed to surrounding tissues |
Etiology |
A small subset present in the setting of familial/multiple cylindromatosis (Brooke-Spiegler) syndrome, an autosomal dominant hereditary disease |
Unknown |
Histology |
Invasive growth is present. The invasive edge is generally seen as rounded lobules with pushing borders (Fig. 1.5.1). Perineural invasion is not common but may be seen
Growth in nests and trabecula with peripheral palisading of tumor nuclei surrounding paler cells in the center (Fig. 1.5.2)
Scattered ducts within the nests of basaloid cells are often present (Fig. 1.5.3)
Tumor cells have minimal cytoplasm, round to oval nuclei with pale chromatin, and indistinct cell borders. They may demonstrate streaming or spindling
The classic cribriform architecture of adenoid cystic carcinoma is absent
In the membranous variant, eosinophilic basal lamina material is deposited within and around the tumor nests (Fig. 1.5.4). At times, this deposition may impart a cribriform pattern
Cellular atypia and mitotic activity varies but is usually minimal
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Highly infiltrative growth. Perineural invasion is a common finding (Fig. 1.5.5)
Mixed growth patterns including tubular, cribriform, and solid (Figs. 1.5.6 and 1.5.7)
The cribriform pattern is the most common, with cylindromatous microcystic spaces (false ducts) filled with basophilic mucoid or hyaline basement membrane-like material (Fig. 1.5.6)
Adenoid cystic carcinomas with a predominant solid growth pattern are most likely to be confused with a basal cell adenocarcinoma (Figs. 1.5.7 and 1.5.9)
The predominant cell type, the myoepithelial cell, has uniformly angulated hyperchromatic nuclei with indistinct nucleoli. The cytoplasm is scant and clear to eosinophilic (Fig. 1.5.8)
Tumor necrosis and elevated mitotic rates are uncommon, but the solid form may exhibit increasing degrees of cellular atypia and mitotic activity (Fig. 1.5.9)
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Special studies |
The tumor is variably positive for p63, S100, actin, calponin, and GFAP, supporting myoepithelial differentiation of some of the cells. C-kit highlights ducts
Negative for MYB-NFIB gene fusion
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Actin, calponin, S100, p63, and p40 highlight the myoepithelial cells, while c-kit and EMA highlight the ductal cells
Approximately 50% of adenoid cystic carcinomas harbor a (6;9) translocation resulting in MYB-NFIB gene fusion
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Treatment |
Surgical excision, usually without radiotherapy or lymph node dissection |
Wide local resection with adjuvant radiotherapy. Routine neck dissection is not usually performed as lymph node metastases are uncommon. Chemotherapeutic agents are generally ineffective |
Prognosis |
Good. It may recur locally but regional and distant metastases are rare |
Patients have a good 5-year survival (75%-80%) but poor 15-year survival (25%-30%) due to slow but relentless growth. Tumors metastasize to lung, bone, liver, and brain. Tumors with a predominantly solid pattern are more aggressive |