NEOPLASTIC DISEASE: BENIGN MASSES

ONCHOCERCIASIS A roundworm larva that causes river blindness. Ninety percent of cases are in Central or West Africa; however, it also can be found in Central and South America. Infections occur in 10to 15-year cycles and are caused by Onchocerca volvulus carried by the Simulium black fly. The fly incubates in aerated, fast-moving water (most infections are in villages within 1 mile of a river); the worm larvae enter the host and travel to subcutaneous nodules (look like buboes). The nodules are mating male and female worms, and larvae are found mainly in the skin but also in the eye. If not taken up by the black fly in a blood meal, the larvae die in the host (straighten and stiffen) and cause sclerosing keratitis, optic neuritis, or choroiditis. To examine, have the patient keep his or her head down for 30 minutes to better see the larvae in the aqueous at the slit lamp. The disease is preventable with ivermectin every 6 months for 10 years, which is a dog heartworm pill that neuters female worms but does not kill the adults.

PARINAUD’S OCULOGLANDULAR SYNDROME Chronic follicular conjunctivitis (lymphoid follicles without germinal centers) with lymphadenopathy; often from Bartonella henselae (cat-scratch disease) or tuberculosis if from endemic area, plus many other causes.

PHTHIRUS PUBIS Crab louse that prefers lashes and pubic hair due to the spacing of cilia; transmitted by sexual contact. Treat by mechanical removal or with bland ointment or pilocarpine gel. Physostigmine ointment is effective as a respiratory poison against the louse but has ocular side effects.

PICORNAVIRUS Enterovirus 70, coxsackievirus; causes hemorrhagic conjunctivitis.

STAPHYLOCOCCUS SPECIES May cause conjunctivitis, keratitis, phlyctenulosis, and marginal corneal infiltrates (hypersensitivity to staphylococcal exotoxins released from staphylococcal blepharitis and meibomianitis with lucent area separating from limbus; use mild steroid drops).

Neoplastic Disease: Benign Masses

AMYLOID Unilateral, yellowish, flat eyelid or conjunctival lesion that hemorrhages when eye is rubbed (amorphous substance laid down like concrete; scratching ruptures the encased blood vessels); usually seen in young females.

COGAN’S PLAQUE Scleral pigment spot around Axenfeld’s nerve loop (melanocytic cyst); benign.

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118 CORNEA AND CONJUNCTIVA

CONJUNCTIVA LYMPHOID HYPERPLASIA Mixed T and B cell polyclonal (heavy and light chains) increase in lymphoid cells; spectrum with conjunctiva lymphoma and appears as fleshy pink mass

DERMATOLIPOMA Choristomas (tissue in an abnormal location)

Lipodermoid, dermal lipoma: yellow-tan nodule composed of adipose tissue. Superotemporal conjunctiva location may extend deep into orbit; thus, excision with caution. If superotemporal, may be associated with Treacher Collins–Franceschetti.

Solid limbal dermoid: keratin-filled nodule with pilosebaceous units and thick epithelium that looks like skin. Characterized by inferotemporal limbus with lipid in adjacent corneal stroma. May cause amblyopia and astigmatism. May be difficult to excise, as it involves Bowman’s membrane and thus scars. Usually sporadic or associated with Goldenhar’s syndrome.

Episcleral osseous choristomas: superotemporal plaque of mature bone; may be complex with other tissue (adipose, cartilage, etc.).

EPIDERMAL INCLUSION CYST (EIC) Conjunctival lesion that often arises after trauma or surgery; usually excise.

BENIGN HEREDITARY INTRAEPITHELIAL DYSKERATOSIS (BHID) Bilateral bulbar leukoplakic lesions without dysplasia; characterized by parakeratosis, dyskeratosis, and hyperkeratosis. Seen in triracial families (Haliwa Indians) from Halifax County, North Carolina.

JUVENILE XANTHOGRANULOMA (JXG) Nonneoplastic histiocytic proliferation in infants that regresses by age 5; characterized by dermatologic vascular anomalies, granulomas, fleshy tan nodules, vascularized iris lesions that may cause heterochromia or spontaneous hyphema, and secondary glaucoma. Touton giant cells and lipid-filled histiocytes are seen on pathology. Treat hyphema with steroids and IOP control.

PYOGENIC GRANULOMA Exuberant growth of granulation tissue with chronic lymphocytic inflammation that arises in areas of trauma or chalazion. Not pyogenic and not a granuloma.

SARCOID GRANULOMA Usually millet-seed granulomas on the conjunctiva with giant cells (epithelioid histiocytes). Diagnosis is a clinicalpathological correlate (no special stains to prove sarcoid); thus, must rule out other granulomatous causes with tissue biopsy for Gram stain, acid-fast stain, silver stain for organisms, and foreign body. Lacrimal gland is most commonly involved ocular tissue, followed by conjunctiva.

SQUAMOUS PAPILLOMA Sessile or pedunculated fleshy growth with fibrovascular core that may be viral or nonviral in origin. Viral cases tend

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to be pedunculated, recur after incomplete excision, and regress spontaneously. May be multiple or recurrent in children or immunocompromised patients (e.g., HIV). Rare malignant potential. Careful when excising to avoid viral spread.

Sessile: seen in older patients, usually HPV 16, 18; higher malignant potential.

Pedunculated: seen in younger patients, most commonly HPV 8, 11.

Neoplastic: Malignant

BASAL CELL CARCINOMA (BCC) In conjunctiva found only on caruncle (only normal conjunctival site with pilosebaceous units).

CONJUNCTIVAL INTRAEPITHELIAL NEOPLASIA (CIN) Thickened epithelium replaced by dysplastic, atypical cells arising from basal layer. Typically limbal leukoplakic lesion in interpalpebral fissure with abrupt transition.

Be suspicious of a lesion that turns from red to white, from flat to elevated, or from bulbar conjunctiva to limbus, and presence of a feeding blood vessel.

Predisposing factors: HPV 16 or 18, older patient, smoker, actinic damage, and petroleum exposure.

Pathology: faulty maturation and polarity, basilar unrest, shouldering, dysplasia, basement membrane intact. Mild if <50% thickness; severe if >50%. Called carcinoma in situ (CIS) if it is full thickness and the BM is intact, or squamous cell carcinoma (SCC) if it violates the BM. Mucoepidermoid variant has poor prognosis.

Diagnose and treat with excisional biopsy with or without cryotherapy. Is a local disease (no metastatic potential, as no blood vessels are in the epithelium), but excise any lesion invading the limbus or cornea or that is leukoplakic.

CONJUNCTIVAL LYMPHOMA ‘‘Salmon patch’’ or ‘‘fish flesh’’ neoplastic growth that arises from resident conjunctiva lymphocytes (i.e., follicles from mucosa-associated lymphoid tissue: no large collections of lymphocytes in the orbit except in the lacrimal gland). Monocellular diffuse B cell monoclonal infiltrate with plasmacytoid (Dutcher) bodies without germinal centers. Biopsy for permanent sections and send fresh tissue for flow cytometry. Treat with radiation therapy.

Most benign of all lymphomas; usually a local disease. Decreasing risk of systemic involvement (lid-orbit-conjunctiva [LOC]): 66% of eyelid lymphoma patients have systemic lymphoma, 33% if orbit is affected, and 20% if conjunctiva is involved.

CONJUNCTIVAL RHABDOMYOSARCOMA Embryonal variant (botryoid

‘‘grapelike’’ appearance).

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120 CORNEA AND CONJUNCTIVA

KAPOSI’S SARCOMA Red lesion usually in HIV/AIDS patients with extravasated blood on pathology. Treatment usually is not necessary unless the lesion is causing a functional problem; can be excised or treated with chemotherapy or radiation therapy.

MULTIPLE ENDOCRINE NEOPLASIA IIB (Also known as MEN III, SippleGorlin syndrome): characterized by enlarged corneal nerves, pheochromocytoma (check urine vanillylmandelic acid [VMA]), medullary thyroid carcinoma (check serum calcitonin), and mucosal neuromas.

ONCOCYTOMA, OXYPHILIC ADENOMA Benign, glandular tumor of caruncle (also found on nasal conjunctiva and lacrimal gland); pathology shows big pink cells with cytoplasm full of mitochondria.

SEBACEOUS CELL CA Pagetoid spread from eyelid into conjunctiva; identified with oil red O stain; best seen with fresh frozen tissue.

SQUAMOUS CELL CA (SCC) Most common malignancy of the conjunctiva, which presents as a leukoplakic lesion, locally invasive but rarely invades the orbit or globe (malignant epithelial ingrowth usually via lymphatics or surgical wound). Pathology: well differentiated, ‘‘pink and below’’ tumor that violates BM, cytokeratinþ. Risks include age, alcohol use, CIN, AIDS, UVB (damages p53 tumor suppressor gene). Excise and consider cryotherapy, or if large, then biopsy, image; may need to exenterate.

Spindle cell variant: aggressive, faster cell turnover (vertical nuclei).

Mucoepidermoid variant: 4 to 6% of SCC; age >60. Very aggressive and invasive, with associated malignant goblet cells; suspect if recurrent after primary excision.

Neoplastic: Melanocytic Lesions

BENIGN, RACIAL, OR OCULAR MELANOSIS, COMPLEXION ASSOCIATED PIGMENTATION (CAP) Seen in darkly pigmented patients with increasing conjunctival pigmentation; bilateral, does not wax/wane; pigment in basal epithelial layer; no malignant potential.

CONJUNCTIVA COMPOUND NEVI Most common pigmented conjunctival lesion. Can rapidly increase in size and pigmentation, especially during puberty and pregnancy. Many are amelanotic (melanocytes are not obligated to produce melanin). Any variant can have cysts. Generally, excise all new nevi in patients over 20 years old.

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