NEOPLASTIC, EYELID: BENIGN CYSTIC LESIONS

may be seen in patients with AIDS. CT may show allergic ‘‘aspergilloma,’’ and pathology shows septate hyphae on H & E. Usually does not invade. Debride sinus, and treat with steroids.

PRESEPTAL CELLULITIS Acute onset of pain, tenderness, erythema, tense swelling but no proptosis, EOM restriction, APD or decreased VA and no pain with eye movement. Most common risk factor is skin trauma; may also be from chalazion, upper respiratory infection, sinusitis, or dental work. Usually from staphylococcus, streptococcus, or Haemophilus influenzae.

Most adults can be diagnosed and treated clinically, but if unsure whether orbital involvement or in pediatric patient, then get complete blood count (CBC), blood culture, and CT.

Oral antibiotics 7–10 days (e.g., Augmentin 500 mg 3 times per day or Keflex 500 mg 4 times per day). If failure to respond, then rule out abcess or foreign body. Sinus drainage if needed, warm compresses for 20 minutes 6 times per day. If <4 years old, hospitalize and use IV antibiotics (neonates are usually nonbacterial as they are protected by maternal Ab, but young children in general are more susceptible because of immunologic naivete, especially against encapsulated organisms due to poor splenic opsonization).

Differential diagnosis: pseudotumor has ‘‘boggy’’ pink skin, not erythematous or indurated; no sinus disease or abscess. If diagnosis is uncertain, okay to admit for IV antibiotics; if no improvement in 48 hours, begin steroid treatment for pseudotumor.

TRACHOMA Chlamydial antigen in conjunctiva causes severe inflammatory reaction, resulting in posterior lamella shortening with entropion, keratitis sicca, and trichiasis. See Chapter 3.

TRICHINOSIS Nematode Trichinella spiralis from poorly cooked pork with encystment of muscles, eosinophilia. Treat with thiabendazole.

Neoplastic, Eyelid: Benign Cystic Lesions

EPIDERMAL INCLUSION CYST (EIC) Unilocular, solitary, slow-growing lesion from sequestered epithelium beneath epidermis, with epithelium cells facing lumen that secrete keratin. Milia are small multiple EIC.

HIDROCYSTOMA (SUDORIFEROUS CYST) Sweat gland origin (eccrine) with clear fluid (transilluminate), unilocular, and on pathology show arborization with cuboidal bilayer facing lumen.

Apocrine hidrocystoma: from Moll’s glands near lid margin; less common, pigmented (blue); arborizing lumen, apical ‘‘snouting’’ typifying apocrine secretion, and double epithelium layer on histology.

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60 ORBIT, EYELIDS, AND OCULAR ADNEXA

SYRINGOMA Most common adnexal eyelid tumor, from ductal eccrine sweat gland origin; benign; yellow, waxy, tadpole appearance. Syringocystadenoma (papilliform).

Neoplastic, Eyelid: Benign Growths

AMYLOID Periorbital cutaneous deposition usually with nonfamilial primary amyloidosis or secondary to systemic plasma cell dyscrasia (rarely with familial primary or other types of amyloidosis).

CHALAZION Obstruction of sebaceous apparatus with foreign body reaction around lipid, sterile, chronic lipogranuloma with multinucleated giant cells. Treat with hot compresses; if not resolved, then incision and curettage. If recurrent, differential diagnosis includes sebaceous cell CA.

HORDEOLUM Internal hordeolum, also called internal chalazion, is usually sterile. The acute external (anterior lamella) Zeis’ glands, stye, is usually from staphylococcal infection.

JUVENILE XANTHOGRANULOMA (JXG) Benign nodular granulomatous disorder with multiple red-orange skin masses; iris xantholomas may cause spontaneous hyphema. Pathology shows Touton giant cells.

KERATOACANTHOMA Form of pseudoepitheliomatous hyperplasia (also seen in inverted follicular keratosis) that is an inflamed nodule with a central ulcer of keratin with very rapid onset and growth in an elderly patient. May resolve spontaneously but is usually excised, as it is difficult to distinguish from squamous cell carcinoma (SCC). Pathology shows ‘‘shoestring’’ sign (if you figuratively pull a string taut across the basement membrane of the histology slide, the lesion is always above the BM).

PHAKOMATOUS CHORISTOMA, ZIMMERMAN’S TUMOR Rare pediatric tumor of lenticular origin (lens in eyelid).

SEBACEOUS ADENOMA Rare benign tumor, seen as small yellow nodules usually on the face.

Muir-Torre syndrome: multiple sebaceous adenomas with 50% incidence of visceral cancer (especially colon). Male to female ratio ¼ 2:1, autosomal dominant with high penetrance; may also have multiple keratoacanthomas.

SQUAMOUS PAPILLOMA, SKIN TAG, ACROCHORDON Sessile or pedunculated proliferation of benign epidermis. Pathology shows apical keratosis with a fibrovascular core and red blood cells in the horn, as well as ‘‘hand in glove’’ appearance. May treat with excision or oral high-dose histamine-2 receptor blockers (e.g., Tagamet 4–5 g/day)

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SEBORRHEIC KERATOSIS (SK) Variant of squamous papilloma with greasy ‘‘stuck-on’’ appearance. Pathology shows basaloid acanthosis, intraepithelial horn cysts, and variable hyperkeratosis. Is not CA, and basal cells are above the plane of the epidermis.

Dermatosis papulosa nigra: multiple pigmented SK; common on facial skin of darkly pigmented patients.

Inverted follicular keratosis: irritated SK, another form of pseudoepitheliomatous hyperplasia (as with keratoacanthoma). Lid margin is the primary body site for inverted follicular keratosis.

VERRUCA VULGARIS, COMMON WART Nontender, elevated, firm papule from human papilloma virus (HPV) infection. Pathology shows elongated acanthotic papillae that look like ‘‘church spires’’ and vacuoles in upper squamous layer that contain virus. Excision with cryotherapy to the base.

XANTHELASMA Symmetric, flat, yellow lesions that typically begin nasally in the upper eyelid. Pathology shows foamy histiocytes in the dermis with extruded lipid engulfed by macrophages. Usually normal serum lipid level (check if the patient is <40 years old) and also associated with diabetes. Excise (although may have deep extension), or use CO2 laser or 100% trichloroacetic acid (TCA). Recurrence common.

Neoplastic, Eyelid: Hair Follicle Tumors (‘‘Tricky’’ Tumors)

TRICHOEPITHELIOMA Most common benign hair follicle neoplasm with fleshy nodules that resemble BCC; usually presents in children and young adults, and may be inherited in autosomal dominant fashion.

TRICHOFOLLICULOMA Rare in periorbital skin but has firm nodule with central pore with white hairs.

TRICHILEMMOMA Nodular or fleshy malignant tumor from outer hair sheath (lemma ¼ rind or sheath).

Cowden disease: multiple facial trichilemmomas associated with occult neoplasm (especially breast or thyroid CA) and other hamartomas (keratoses, oral papillomas); autosomal dominant.

Neoplastic, Eyelid: Premalignant Lesions

ACTINIC KERATOSIS Most common precancerous lesion, with scaly papillary appearance. Pathology shows solar elastosis like pterygium; also has cellular atypia (but no anaplasia like CA), and demonstrates parakeratosis. Twelve percent become CA. Treat with cryotherapy, or excise with topical 5-fluorouracil (5-FU).

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62 ORBIT, EYELIDS, AND OCULAR ADNEXA

BOWEN’S DISEASE Is full thickness carcinoma in situ or intraepidermal SCC. Larger lesion than actinic keratosis, solitary; usually sun-exposed skin with ‘‘windblown look’’ on histology. If on non-sun-exposed skin, consider occult cancer (Rulon-Helwig syndrome) or arsenic ingestion.

Neoplastic, Eyelid: Malignant Lesions

BASAL CELL CARCINOMA (BCC) Most common lid cancer (87% of eyelid CA). Primary site is lower lid (50–60%), followed by medial canthus (25– 30%,) and upper lid (10–15%). From UV exposure and solar keratosis. Often underestimated size by its appearance; great masquerader (like sebaceous gland CA). Early signs are telangiectasia and skin smoothing.

Nodular is the most common type, with pearly raised nodule with central umbilication. May also present as nodulo-ulcerative, adenoid, pigmented, superficial, keratotic, cystic, or follicular, all of which are more benign. Infiltrating, morpheaform, or sclerosing/fibrosing types are less common but much more aggressive, often subclinical, increased recurrence; may have lash loss.

Pathology shows ‘‘blue and below’’ (bluish-colored tumor cells that are below the BM), retraction artifact, peripheral palisading (also seen with other adnexal cancers), and desmoplasia. Morpheaform variant also has peripheral radiating cords (‘‘Indian filing’’).

Tumors at high risk for recurrence: sclerosing BCC and poorly defined tumors, ‘‘H’’ zone of face (down both temples and cheeks and across nose), cosmetically sensitive areas, young age, large size, digit or medial canthus location, and prior recurrent CA.

Treat with excision, radiation therapy, or cryotherapy. May recur locally; unlikely to metastasize but can have perineural invasion. Medial canthus area may have a worse prognosis with a dumbbellshaped tumor that extends into orbit because septum is discontinuous due to spiral configuration of the bone and because surgeons undercut while trying to spare the canalicular system (thus, may send to Mohs’ surgeon, who may have less regard for the lacrimal drainage system). If medial canthus is involved, avoid DCR for at least 1 year to rule out recurrence.

Basal cell nevus syndrome (Gorlin’s syndrome): autosomal dominant with multiple BCC associated with cardiac, sternal, and other bony abnormalities ( jaw cysts, bifid ribs). Do not give radiation therapy.

ERDHEIM-CHESTER DISEASE Rare multisystem disease characterized by lipogranulomas in the liver, heart, kidneys and retroperitoneal fibrosis. May have bilateral xanthelasma-like eyelid lesions or proptosis from orbital masses or bony lesions. Pathology shows Touton giant cells. Poor prognosis.

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KAPOSI’S SARCOMA Red-blue dermal mass usually in HIV patients. Spindle-cell tumor from endothelial cell origin with extravasated blood in slitlike spaces on pathology. Excise.

LACRIMAL SAC TUMORS Signs include epiphora, irreducible swelling of the sac (classically above the MCT), bleeding with probing, and secondary dacryocystitis. Primary malignancies are rare but are most often papillary carcinoma; may also be squamous cell, transitional cell, or mixed cell type. Treat with local excision. Second most common lacrimal sac tumors are lymphomas; treat with radiation therapy after biopsy and systemic evaluation.

MERKEL CELL CARCINOMA Rare, highly malignant red ‘‘plum’’ tumor in older patient that may mimic chalazia and spreads via lymphatics. Pathology looks like small-cell lung CA, from neuroendocrine, amine precursor uptake and decarboxylation (APUD) cell. Wide excision with radiation therapy.

NECROBIOTIC XANTHOGRANULOMA Older patients with nodular yellow, inflamed skin lesion (may be mistaken for xanthelasma), with Touton multinucleated giant cells full of lipid and foci of necrosis on pathology. Twenty-six percent have plasma cell malignancy.

PILOMATRIXOMA, BENIGN MALHERBE’S CALCIFYING EPITHELIOMA, PILOMYXOMA, MATRIXOMA Calcified epithelioma of childhood that presents as a solitary mobile, firm, blue-red nodule with hair cortex and basophilic epithelial cells and shadow cell calcification. May be multiple on upper arms.

SEBACEOUS ADENOCARCINOMA 3% of eyelid CA that arises from meibomian glands; thus, primary site is upper lid (two thirds of cases), followed by lower lid. Yellow color, diffuse mass; a great masquerader that may present as recurrent chalazion or unilateral blepharitis with madarosis. Pagetoid spread may cause conjunctival fibrosis like OCP or Arlt’s line in adult inclusion conjunctivitis.

Older patients: 15% mortality due to delayed diagnosis; rare in other body sites (thus, general pathologists often not familiar with this diagnosis).

Pathology: bizarre atypical cells with frothy cytoplasm, desmoplastic reaction. May be BRST-1 positive (breast tumor marker) especially with pagetoid spread.

Full-thickness biopsy; submit in formalin for fat stains (Oil Red O), and get frozen sections to establish margins.

Treat with wide excision, possible exenteration.

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