Colleen Halfpenny

BASICS

DESCRIPTION

Benign conjunctival lesions are a group of tumors, including papilloma (Pap), Kaposi’s sarcoma (KS), limbal dermoid (LD), sarcoidosis (Sarc), and pyogenic granuloma (PG) with a low potential for malignant growth and a wide variety of etiologies and management considerations.

EPIDEMIOLOGY

Incidence

• Systemic KS: 4–14% in ocular adnexa (1)[C]

• Ocular KS: 7–18%

• Ocular Sarc: 7–19%

RISK FACTORS

• Pap: Mother with positive human papillomavirus (HPV) history, UV radiation

• KS: Immunocompromised states, including HIV/AIDS, chemotherapy, organ transplantation, elderly males

• LD: <18 years of age

• Sarc: African American race

• PG: transconjunctival surgical incisions (strabismus, retinal surgery), trauma (2)[B]

Genetics

Sarc: HLA-Bw15, HLA-B8, HLA-B13

PATHOPHYSIOLOGY

• Pap: HPV, a double-stranded DNA virus transmitted via direct contact, often through passage of infected birth canal leading to proliferation of fibrovascular connective tissue, acanthosis, and hyperkeratosis

• KS: Dysregulated inflammatory cytokine response due to human herpesvirus-8

• LD: Congenital choristomatous changes due to an unknown process; classified based upon the extent of anterior segment involvement

• Sarc: Inflammatory hyperactivity due to dysregulated cytokine responses following an antigenic stimulus

• PG: Proliferative fibrovascular and inflammatory response usually secondary to aberrant wound healing

ETIOLOGY

• Pap: HPV, most commonly benign types 6 and 11, rarely type 16 or 45

• KS: Human herpesvirus-8 (HHV-8) DNA or Kaposi’s sarcoma-associated herpesvirus (KSHV), implicated with patients who are HIV-negative and HIV-positive

• Sarc: Unknown etiology. Suggested causes: Environmental toxins, infectious agents, genetic transmission

• LD: Unknown

• PG: Usually due to prior inflammatory condition or status post trauma or surgery

COMMONLY ASSOCIATED CONDITIONS

• LD: Goldenhar’s syndrome

• Sarc: Mikulicz’s syndrome: Salivary and lacrimal gland enlargement with keratoconjunctivitis sicca

DIAGNOSIS

HISTORY

• Pap, KS, LD, Sarc, PG:

– often asymptomatic

– mass effect symptoms

– foreign body sensation

– itching

– tearing

– mucous

– ptosis

– photophobia

– blurry vision

– swelling

– trichiasis

– poor lid apposition

– cosmetic effects

• KS: Immune status

• Sarc: Lung and skin nodules, dyspnea, cough malaise, fatigue, lymphadenopathy

• LD: <18 years of age, enlarging since birth, especially after puberty

• PG: History of surgery, inflammatory condition

PHYSICAL EXAM

• Pap: Pedunculated or sessile, pink-red, fleshy, smooth often verrucous appearing on bulbar or palpebral conjunctiva. Viral usually multiple lesions in inferior fornix; single lesions may suggest nonviral etiology and may extend toward the limbus.

• KS: Flat or raised, reddish-purple, oval, nontender mass, or nodular, well-circumscribed lesion

• Sarc: Single or multiple, yellow or salmon colored nodules. Most commonly on palpebral conjunctiva of lower cul-de-sac or plica semilunaris

• LD: Yellowish-white oval-shaped, solid mass involving bulbar conjunctiva, typically near the inferotemporal corneoscleral limbus; may have fine hairs

• PG: well-vascularized, elevated, red-fleshy mass with fibrous stalk

• Secondary findings for all of the above include chemosis, mechanical ectropion, superficial punctuate keratopathy, and corneal dellen.

DIAGNOSTIC TESTS & INTERPRETATION

Lab

• KS:  HIV ELISA, HIV Western Blot, CD4

• Sarc: Angiotensin-converting enzyme, calcium, magnesium, phosphorous

Imaging

Sarc: Chest X-ray

Diagnostic Procedures/Other

• Slit lamp, dilated fundus examination, tonometry

• Pap, KS, Sarc, PG: incisional or excisional biopsy, if suspicious for malignancy

• LD: Depth of corneal involvement can be evaluated with ultrasound biomicroscopy (UBM). Examine patient for signs and symptoms of Goldenhar’s syndrome, including preauricular skin appendages, hearing loss, lid coloboma, and naso-oro-vertebral anomalies (4)[C].

Pathological Findings

• Pap: Multiple branching fronds with a fibrovascular core lined by thickened, acanthotic, squamous epithelium with or without koilocytosis

• KS: Thin, dilated, endothelial-lined vascular channels surrounded by spindle cells and inflammatory reaction

• LD: Conjunctival epithelium with epidermal appendages surrounding simple choristomatous tissue including hair follicles, sebaceous glands, muscle, adipose, teeth, bone, and cartilage

• Sarc: Noncaseating granulomas

• PG: Granulation tissue, chronic inflammation, small blood vessels

DIFFERENTIAL DIAGNOSIS

• Pap: Conjunctival intraepithelial neoplasia, squamous cell carcinoma, amelanotic melanoma, lymphoma

• KS: Subconjunctival hemorrhage, hemangiopericytoma, pyogenic granuloma, capillary and cavernous hemangioma, lymphoma, malignant melanoma, pingueculitis, bacillary angiomatosis

• Sarc: Chalazia, pyogenic granuloma, conjunctival intraepithelial neoplasia, mycobacterial infections, fungal infection, foreign body granuloma, papilloma

• DL: Pterygium, pinguecula, juvenile xanthogranuloma, staphyloma, foreign body reaction, sclerocornea

• PG: Foreign body granuloma, chalazion, sarcoidosis, papilloma, lymphoma

TREATMENT

MEDICATION

First Line

• Pap: Observation for spontaneous resolution

• LD: Topical lubrication, removal of irritating hairs

• KS: Highly active antiretroviral therapy (HAART) (1)[B]

• Sarc: Topical steroid

• PG: Observation for spontaneous resolution

Second Line

• Pap: Topical interferon-alpha-2b, mitomycin C, oral cimetidine (3)[C]

• KS: Local chemotherapy (vinblastine and vincristine), low dose radiotherapy, topical interferon-alpha, human choriogonadotropin

• PG: Low-dose plaque radiotherapy for recurrent PG (4)[C]

ADDITIONAL TREATMENT

Issues for Referral

LD: If clinically suspicious for Goldenhar’s syndrome, consult pediatrician with genetics specialty

SURGERY/OTHER PROCEDURES

• Pap: Excisional biopsy with or without cryotherapy if symptomatic, or if concern for malignancy

• KS: Surgical excision, if refractory to HAART, with 1–2 mm margins

• LD: Superficial sclerokeratectomy with or without lamellar graft

• Sarc: Surgical excision, if symptomatic

• PG: Surgical excision, if symptomatic

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Outpatient care for periodic observation of lesions and surgical excision of benign conjunctival masses

• Inpatient care for systemic management of complicating illness

PROGNOSIS

Highly favorable prognosis

COMPLICATIONS

• Lesions may reoccur after excision

• Secondary to surgical excision, including scaring, infection, bleeding, blindness

• LD: Refractive error; cornea may remain opacified from scar after surgery

• Systemic complications of underlying conditions

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