Carol L. Shields

BASICS

DESCRIPTION

• Most common conjunctival tumor

• Represents 28% of all conjunctival tumors and over 50% of all melanocytic conjunctival tumors

• Can be melanotic (∼80%) or amelanotic (∼20%)

• Can be congenital (present at birth or within the first 6 months of life) or acquired

• If acquired, usually clinically visible by the first or second decade of life

• Can become more pigmented over time, especially during hormonal changes, such as puberty and pregnancy

Pediatric Considerations

• An increase in size may be seen in young children.

• Increased pigmentation may be seen during puberty.

Pregnancy Considerations

Increased pigmentation may be seen during pregnancy in cutaneous nevi, but this has not been well documented in conjunctival nevi.

EPIDEMIOLOGY

Occurrence is most common in Caucasians (89%); less frequently seen in African Americans (6%) and Asians, Hispanics, or Indians (5%).

RISK FACTORS

Ethnicity: Non-Hispanic whites.

GENERAL PREVENTION

Most conjunctival nevi occur at the nasal and temporal limbus in the sun exposed regions of the eye. Avoidance of sun exposure might reduce conjunctival nevus development, but more importantly, avoidance of sun exposure might reduce transformation of conjunctival nevus into melanoma.

COMMONLY ASSOCIATED CONDITIONS

• Generally sporadic without systemic associations

• Rare association with the Carney complex and the dysplastic nevus syndrome

DIAGNOSIS

HISTORY

Patients may be asymptomatic (10%) or may report noticing a “spot” on the eye (88%), inflammation (3%), or rarely pain (<1%) (1)[C].

PHYSICAL EXAM

• Usually presents as a discrete, variably pigmented, slightly elevated conjunctival mass.

• The mass is within the conjunctiva and moves with conjunctival displacement.

• Nevus typically occurs on the bulbar conjunctiva (∼70%), most commonly in the interpalpebral zone (∼85%) (1,2)[C].

• May be pigmented (51%), partially pigmented (28%), or completely amelanotic (21%) (2)[C].

• Multiple, intralesional clear cysts can be seen on slit-lamp biomicroscopy (1,2)[C].

• If located on the bulbar conjunctiva, conjunctival nevi typically abruptly stop at the limbus and do not involve the cornea (1)[C].

• In the bulbar conjunctiva, temporal (46%) or nasal (44%) location is more common than superior (6%) or inferior (%5) location (1)[C].

DIAGNOSTIC TESTS & INTERPRETATION

Imaging

Initial approach

• Slit-lamp photography

• Anterior segment optical coherence tomography to document cysts

Follow-up & special considerations

Serial photography every 6–12 months to rule out growth/malignant transformation.

Diagnostic Procedures/Other

Anterior segment optical coherence tomography.

Pathological Findings

• Diffuse infiltration or distinct nests of benign melanocytes near the basal layer of the epithelium

• Most often classified as junctional (small nest of melanocytes in the basal layer of the epithelium), compound (cell migration into the underlying stroma), or subepithelial/deep (cell migration entirely into the stroma)

• Caveat: In general, the melanoma-specific antigen (HMB-45) shows a positive reaction with conjunctival nevus and melanoma and can therefore not be used reliably to differentiate the two conjunctival lesions.

DIFFERENTIAL DIAGNOSIS

• Primary acquired melanosis

• Racial melanosis

• Secondary melanosis

• Malignant melanoma (melanotic & amelanotic)

• If amelanotic

– Inflamed pinguecula

– Conjunctival cyst

– Allergic conjunctivitis

– Foreign body granuloma

– Episcleritis

– Squamous epithelial neoplasia

– Papilloma

– Lymphoma

– Lymphangioma

TREATMENT

MEDICATION

None available

ADDITIONAL TREATMENT

General Measures

Initial management for a small, typical conjunctival nevus is periodic observation with photographic documentation (2)[C].

Issues for Referral

• Any diagnostic uncertainty

• When surgical excision is considered

SURGERY/OTHER PROCEDURES

• Excisional biopsy using the “no touch” technique whereby the tumor is never manipulated should be used. This is approached using a dry ocular surface without balanced salt solution. The cornea is dried and absolute alcohol on a Weck sponge is precisely applied to the limbus immediately adjacent to the nevus without spillage to other sites. The corneal epithelium is removed. The conjunctival mass is removed with Westcott scissors and a 3–4 mm margin. After removal, cryotherapy is applied to the remaining conjunctival margins. Closure is done with absorbable sutures.

• Consider complete excisional biopsy if

– Suspicious changes

– Documented growth

– Location in the forniceal or tarsal conjunctiva

– Limbal location with corneal involvement

– Feeder vessels

– Intrinsic vessels

– Larger lesion without cysts

– Family history of conjunctival or cutaneous melanoma

– Distinct onset in middle age or later life

– Recurrence of a previously excised lesion

– Ocular irritation

– Cosmetic concern

– Cancerophobia

• In general, incisional biopsies are contraindicated in lesions that can be resected entirely in one procedure.

IN-PATIENT CONSIDERATIONS

• None required

• Outpatient surgery and management

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Annual ophthalmologic examinations if lesion is typical and nonsuspicious in appearance.

Patient Monitoring

Slit-lamp photography and anterior segment optical coherence tomography every 6–12 months to monitor stability.

DIET

No dietary restrictions.

PATIENT EDUCATION

• See related websites.

• Avoid excessive sun exposure.

PROGNOSIS

Excellent in most cases.

COMPLICATIONS

Irritation and/or dry eye occasionally seen after excisional biopsy.

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