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Adenoid cystic carcinoma (ACC) is a rare, slow-growing but locally aggressive malignant tumor that arises from minor salivary glands, including those located in the nasal cavity, paranasal sinuses, and upper aerodigestive tract.
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It is known for its Perineural spread (PNS) , local recurrence, and delayed distant metastasis.
Epidemiology
- Accounts for <5% of sinonasal malignancies
- Peak incidence: 5th–6th decades
- Slight female predominance
- Most commonly arises in:
- Maxillary sinus
- Nasal cavity
- Ethmoid sinus
Etiopathogenesis
- Originates from mucoserous glands of respiratory mucosa
- Subtypes (based on histology):
- Cribriform (most common)
- Tubular
- Solid (worst prognosis)
Key Pathologic Features:
- Perineural invasion
- Bone infiltration
- Late hematogenous metastasis (e.g., to lungs)
Clinical Features
- Slow progression but extensive local invasion
- Unilateral nasal obstruction