OcuLearning › Adnexa
Lacrimal Gland Mass/Chronic Dacryoadenitis
URGENT — Adnexa
Symptoms
progressive swelling of the outer one-third of the upper eyelid
pain (highly suggestive of malignancy or acute infection)
double vision due to mass effect on extraocular muscles
inferomedial globe displacement ("down and in")
v1/v2 numbness (forehead/temple) suggests perineural invasion by carcinoma
Clinical Findings
Clinical signs
- s-shaped upper eyelid deformity: classic for lacrimal gland enlargement
- "down and in" proptosis (inferomedial displacement)
- "salmon patch" subconjunctival extension suggests lymphoma (malt)
Imaging discriminators (CT/MRI)
- pleomorphic adenoma: painless, >1 year history, fossa remodeling without erosion
- adenoid cystic carcinoma (acc): painful, subacute growth, frank bony erosion
- bilateral enlargement: suspect igg4-rd, sarcoidosis, or sjögren syndrome
- lymphoma: lesion typically "molds" to the globe and conforms to orbital anatomy
Etiology
Epithelial tumors
- pleomorphic adenoma: benign mixed tumor; most common epithelial tumor
- adenoid cystic carcinoma (acc): most common malignant epithelial histology
Inflammatory / systemic
- sarcoidosis: often bilateral and painless; may have lung or skin signs
- igg4-related dacryoadenitis: increasingly recognized systemic inflammatory cause
- idiopathic orbital inflammation (ioi): usually more acute and painful
Other
- lymphoma: often indolent; malt is the most common subtype
- dacryops: simple lacrimal gland ductal cyst
Red Flags
pain or rapid growth (strongly suggests adenoid cystic carcinoma)
bony erosion on ct (indicator of malignancy)
v1/v2 numbness (sign of perineural invasion into the forehead or temple)
bilateral involvement (mandates systemic workup for igg4, sarcoid, or lymphoma)
Investigations
Imaging
- orbital ct (thin-cut): best to evaluate for bony remodeling vs. frank erosion
- chest ct: to screen for sarcoidosis or primary systemic malignancy
Laboratory
- ace, lysozyme, and chest imaging for sarcoidosis
- serum igg4 levels, c-anca/p-anca, and cbc with differential
Biopsy protocol
- STRICT RULE: do NOT perform incisional biopsy on suspected pleomorphic adenoma (seeds tumor and risks malignancy); excise en bloc
- biopsy with flow cytometry is indicated for suspected lymphoma or inflammatory disease
Treatment
Benign epithelial
- pleomorphic adenoma: complete surgical en bloc excision (capsule must remain intact)
Malignant epithelial
- acc: radical surgical resection often with involved bone + adjunctive radiation
Inflammatory / systemic
- sarcoidosis and igg4-rd: systemic corticosteroids or steroid-sparing agents
- lymphoma: localized radiation or systemic chemotherapy depending on subtype
Clinical Pearl
A pleomorphic adenoma of the lacrimal gland must be excised en bloc — incisional biopsy seeds tumor into orbit and virtually guarantees recurrence with malignant transformation risk.
Differential Diagnosis
- sarcoidosis
- orbital lymphoma
- pleomorphic adenoma
- adenoid cystic carcinoma
- acute bacterial dacryoadenitis