Persistent or progressive swelling of the outer one-third of the upper eyelid. Pain or double vision may be present.

Critical

Chronic eyelid swelling, predominantly in the outer one-third of the upper eyelid, with or without proptosis and displacement of the globe inferiorly and medially. Pain may be present, especially in cases of acute IOIS of the lacrimal gland. Erythema is less common. A dull, aching pain over the forehead or along the temple is an ominous sign, suggestive of malignancy.

Other

A palpable mass may be present in the outer one-third of the upper eyelid. Extraocular motility may be restricted. May have conjunctival injection.

• Sarcoidosis: May be bilateral. Typically painless. May have concomitant lung, skin, or renal disease. Lymphadenopathy, parotid gland enlargement, or seventh cranial nerve palsy may be present. Of note, intraocular involvement is uncommon in patients with adnexal sarcoidal inflammation, and vice versa. More common in Americans of African descent, West Africans, and Northern Europeans.

• IOIS: See 7.2.2, Idiopathic Orbital Inflammatory Syndrome. Chronic, painless lacrimal gland enlargement is possible, but atypical for IOIS.

• IgG4-related dacryoadenitis. See Note in 7.2.2, Idiopathic Orbital Inflammatory Syndrome.

• Infectious: Enlarged palpebral lobe with surrounding conjunctival injection. Purulent discharge with bacterial dacryoadenitis, which is much less common than noninfectious dacryoadenitis. Bilateral lacrimal gland enlargement may be seen in patients with viral illnesses. CT scan may show fat stranding, abscess.

• Benign mixed tumor (pleomorphic adenoma): Slowly progressive, painless proptosis or inferomedial displacement of the globe in middle-aged adults. Usually involves the orbital lobe of the lacrimal gland. CT may show a well-circumscribed mass with pressure-induced remodeling and enlargement of the lacrimal gland fossa. No true bony erosion occurs (Figure 7.6.1A).

  Figure 7.6.1: CT and MRI of lesions involving or near the lacrimal gland.

  A: Pleomorphic adenoma with smooth, pressure induced changes in the lacrimal gland fossa (arrows). B: Adenoid cystic carcinoma with bone destruction (arrows) and intralesional calcifications. C: Lymphoma involving the lacrimal gland with molding to the globe. D: Dermoid cyst arising from the frontoethmoidal suture.

  

• Lymphoproliferative tumor: Slowly progressive proptosis and globe displacement in an adult. May have a pink “salmon-patch” area of subconjunctival extension. CT usually shows a lacrimal gland lesion that conforms to the native anatomy and is well circumscribed. Indolent forms spare the bone, but bony erosion may be seen in aggressive histopathology (e.g., diffuse large B-cell and mantle cell lymphoma) (Figure 7.6.1C).

• ACC: Subacute onset of pain over 1 to 3 months, proptosis, and diplopia, with variable progression. Globe displacement, ptosis, and a motility disturbance are common. This malignant lesion often exhibits perineural invasion, resulting in pain along the temple or forehead and intracranial extension. CT shows an irregular mass, often with bony erosion (Figure 7.6.1B).

• Malignant mixed epithelial tumor (pleomorphic adenocarcinoma): Occurs primarily in elderly patients, acutely producing pain and progressing rapidly. May develop primarily or secondarily within a long-standing benign mixed epithelial tumor (“carcinoma ex pleomorphic adenoma”), or incompletely resected benign mixed tumor. CT findings are similar to those for ACC.

• Lacrimal gland cyst (dacryops): Usually an asymptomatic mass that may fluctuate in size. Typically occurs in a young adult or middle-aged patient.

• Others (may not involve the lacrimal gland, but occur superolaterally in the area of the lacrimal gland and fossa): GPA (formerly Wegener granulomatosis), tuberculosis, leukemia, mumps, mononucleosis, syphilis (exceedingly rare), mucoepidermoid carcinoma, plasmacytoma/multiple myeloma, eosinophilic granuloma, metastasis (especially prostate adenocarcinoma), and dermoid cyst (Figure 7.6.1D) (see Tables 7.4.1.2 and 14.3.2).

NOTE Primary, epithelial neoplasms are almost always unilateral; inflammatory disease may be bilateral. Lymphoma is more commonly unilateral, but may be bilateral.

1. History: Determine the duration of the abnormality and rate of progression. Associated pain, tenderness, or double vision? Weakness, weight loss, fever, or other signs of systemic malignancy? Breathing difficulty, skin rash, or history of uveitis (sarcoidosis)? Any known medical problems? History of lacrimal gland biopsy or surgery?

2. Complete ocular examination: Specifically look for keratic precipitates, iris nodules, posterior synechiae, and old retinal periphlebitis from sarcoidosis. As noted, intraocular sarcoidosis is uncommon in patients with ocular adnexal sarcoidosis, but may occur.

3. Orbital CT (axial, coronal, and parasagittal views). MRI is rarely required unless an intracranial extension is suspected. CT is helpful in defining bony anatomy and abnormality.

4. Consider a chest CT, which may diagnose sarcoidosis, primary malignancy, lymphoproliferative disease, metastatic disease, and, rarely, tuberculosis.

5. Consider CBC with differential, ACE, cANCA, pANCA, SPEP, LDH, IgG4/IgG levels, and purified protein derivative (PPD) or IGRA (e.g., QuantiFERON Gold) if clinical history suggests a specific etiology.

6. Lacrimal gland biopsy (see Note below) is indicated when a malignant tumor is suspected, or if the diagnosis is uncertain. If possible, avoid treatment with corticosteroids until a biopsy is obtained.

7. Systemic workup by an internist or hematologist/oncologist when lymphoma or other blood dyscrasia is confirmed (e.g., abdominal and head CT scan, PET/CT scan, possible bone marrow biopsy).

NOTE Do not perform an incisional biopsy on lesions thought to be a benign mixed tumor (pleomorphic adenoma) or dermoid cyst. Incomplete excision of a pleomorphic adenoma may lead to a recurrence with or without malignant transformation. Rupture of a dermoid cyst may lead to a severe inflammatory reaction. These two lesions should be completely excised without violating the capsule or pseudocapsule.

NOTE If ACC is suspected, some experts recommend avoiding large, debulking biopsies for the preservation of the lacrimal artery. A recent study on the treatment of ACC with an intra-arterial chemotherapeutic protocol concluded that efficacy is compromised if the lacrimal artery is not intact. To avoid iatrogenic injury to the artery, perform an anterior biopsy to confirm the diagnosis of ACC. Other experts do not utilize intra-arterial chemotherapy and proceed with complete gross excision of the tumor and involved bone in anticipation of adjunctive radiation therapy.

1. Sarcoidosis: Systemic corticosteroids or low-dose antimetabolite therapy. See 12.2, Intermediate Uveitis, 12.3, Posterior Uveitis and 12.4, Panuveitis.

2. IOIS: Systemic corticosteroids. See 7.2.2, Idiopathic Orbital Inflammatory Syndrome.

3. IgG4-related disease: Systemic corticosteroid therapy or low-dose antimetabolite therapy. Biologic therapy may also be used.

4. Benign mixed epithelial tumor (pleomorphic adenoma): Complete surgical removal.

5. Dermoid cyst: Complete surgical removal.

6. Lymphoma confined to the lacrimal gland: Depends on the subtype of lymphoma. Indolent lesions respond well to radiation therapy alone. Aggressive lesions, even when isolated, typically necessitate systemic chemotherapy, including biologic agents (e.g., rituximab). See 7.4.2, Orbital Tumors in Adults.

7. ACC: Consider pretreatment with intra-arterial cisplatinum, followed by wide excision. Orbital exenteration and craniectomy are used less frequently, especially in smaller lesions, since there appears to be no prognostic advantage over more localized excision followed by radiotherapy. Adjunctive radiation is recommended in all patients, possibly with systemic chemotherapy. Proton beam radiotherapy is offered by some centers, but, to date, there is no proven benefit over conventional stereotactic radiotherapy. Regardless of the treatment regimen, the prognosis is guarded and recurrence is the rule. There is no clear evidence that any specific treatment regimen improves survival. Survival appears to be most dependent on the specific tumor subtype (basaloid or nonbasaloid) and initial tumor size.

8. Malignant mixed epithelial tumor: Similar as for ACC.

9. Lacrimal gland cyst: Excise if symptomatic.

Depends on the specific cause.