Symptoms, imaging findings, treatment methods, and prognosis of adenoid cystic carcinoma (ACC), the most common malignant tumor of the lacrimal gland

 

👁 Adenoid Cystic Carcinoma (ACC) – The Most Aggressive Malignancy of the Lacrimal Gland

Adenoid cystic carcinoma (ACC) is the most common malignant epithelial tumor of the lacrimal gland.
Though it accounts for only 12.5% of lacrimal gland tumors and 1.25% of orbital tumors, it has a notoriously poor prognosis.

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✅ Clinical Presentation

• Occurs primarily in individuals in their 40s
• Originates from the superotemporal orbit, displacing the globe inferomedially
• Often presents with pain, unrelated to eye movement
• Periorbital sensory loss may occur due to nerve infiltration
• Symptoms progress rapidly over months

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✅ Imaging Characteristics

• Poorly defined margins on CT
• Evidence of bony invasion, destruction, and calcification
• Suggests infiltrative malignant behavior

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✅ Treatment & Prognosis

• Requires orbital exenteration with wide surgical margins
• Even after surgery, local recurrence and distant metastasis (brain, lungs, lymph nodes) are common
• 15-year survival rate is less than 10%, highlighting the need for early diagnosis

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✅ Comparison with Pleomorphic Adenoma

FeaturePleomorphic AdenomaAdenoid Cystic Carcinoma

Nature	Benign	Malignant
Growth	Slow (over a year)	Rapid (within months)
Pain	Typically absent	Often present
Imaging	Well-defined borders	Infiltrative, bony invasion
Treatment	Complete excision	Orbital exenteration
Prognosis	Favorable	Poor (high recurrence & metastasis)