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Conjunctival tumors in childrenCarol L. Shields and Jerry A. Shields

Purpose of review

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rieallhoristomas, consisting of displaced tissue elementsnormally not found in these areas. A simple choristomacomprises one tissue element such as epithelium,

351t findings

ost common malignancies include conjunctival

ma and melanoma. In our experience, the most

on conjunctival tumors in children include nevus

, dermolipoma (5%), lymphangioma (3%) and

ry hemangioma (3%). Conjunctival nevi can manifest

arkly pigmented (65%), lightly pigmented (19%) and

etely nonpigmented (16%) mass. Most nevi occurred

nasal or temporal limbus, without involvement of the

. Occasionally, they are located in the caruncle, but

are nevi found in the fornix or tarsal conjunctival

e. Intralesional cysts are visible in 65% of nevi.

e in nevus color over time has been noted in 5% of

and change in nevus size has been documented in

volution of conjunctival nevus into malignant

ma is extremely low (

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whereas a complex choristoma represents variable com-binations of ectopic tissues such as bone, cartilage andlacrimal gland.

Dermoid

Conjuncyellow-wconjuncporally arare casein othedermoidcan becornea,dermoid

The concan bepatient

preauricular skin appendages, hearing loss, eyelid colo-boma, orbitoconjunctival dermolipoma and cervicalvertebral anomalies. Histopathologically, the conjuncti-

ermstseliles

mrva. Ins,un

uceacrereneefra

352 Pediatrics and strabismus

Table 1 Clinical diagnostic categories of conjunctival tumors in262 children [12]

Classification of tumors Number of patients (%)

Choristomatous 26 (10)Benign epithelial 5 (2)Premalignant and malignant epithelial 1 (

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not chana lamellong-ter

Dermolip

Dermolcally redetectejunctivasoft, flu(Fig. 2)bulbar c

Dermolputed to(MRI).epithelivariableadiposetissue mrequirecosmetithe entival fornof the leprolapse

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Conjunctival tumors in children Shields and Shields 353

Figure 2 Dermolipoma in superotemporal conjunctival fornix Figure 3 Epibulbar osseous choristoma on bulbar conjunctivasuperotemporally, presenting as a firm, palpable massge. When the lesion involves the central cornea,lar or penetrating keratoplasty is necessary andm amblyopia should be anticipated.

oma

ipoma is believed to be congenital, but it classi-mains asymptomatic for years and might not bed until adulthood. It typically occurs in the con-l fornix superotemporally and appears as a yellow,ctuant mass with fine white hairs on its surface. It can extend into the orbital fat and onto theonjunctiva, sometimes reaching the limbus.

ipoma has features similar to orbital fat on com-mography (CT) and magnetic resonance imagingHistopathologically, it is lined by conjunctivalum on its surface and the subepithelial tissue hasquantities of collagenous connective tissue andtissue. Pilosebaceous units and lacrimal glandight be present. The majority of dermolipomasno treatment, but larger ones or those that arecally unappealing can be managed by excision ofre orbitoconjunctival lesion through a conjuncti-

Lacrim

Lacreredmassof threprenatiolacriminflambe cytionusua

Comp

Theistomtype,a gresuchclinicsurfaarounht Lippincott Williams & Wilkins. Unauthorized

iceal approach or by removing the anterior portionsion in a manner similar to that used to removed orbital fat.

r osseous choristoma

ar osseous choristoma is a firm deposit of bone,located in the bulbar conjunctiva superotempo-] (Fig. 3). It is believed to be congenital andremains undetected until palpated by the olderOn ultrasonography or CT, the mass demon-

a calcium component. This tumor is usuallyd by observation. Occasionally, a foreign-bodyn necessitates excision of the mass using atival forniceal incision followed by dissection ofor to bare sclera.

The comnevus ssebaceosebaceofeaturesnoidal cof this sand posplex chObservamembra

Epitheli

Severalsquamogland choristoma

l gland choristoma is a congenital lesion, discov-young children as an asymptomatic pink stromalpically in the superotemporal or temporal portionconjunctiva. It is speculated that this lesionts small sequestrations of the embryonic evagi-f the lacrimal gland from the conjunctiva. Thegland choristoma can masquerade as a focus ofation due to its pink color. Rarely, this mass canc due to ongoing secretions if there is no connec-the conjunctival surface. Excisional biopsy is

performed to confirm the diagnosis.

choristoma

njunctival dermoid and epibulbar osseous chor-re simple choristomas, as they contain one tissuech as skin or bone. A complex choristoma containsr variety of tissue derived from two germ layers,lacrimal tissue and cartilage. It is variable in itsappearance and can cover much of the epibulbaror it may form a circumferential growth patternthe limbus. reproduction of this article is prohibited.

plex choristoma has an association with the linearebaceous of Jadassohn [15] (Fig. 4). The nevusus of Jadassohn includes cutaneous features withus nevus in the facial region and neurologic, including seizures, mental retardation, arach-yst and cerebral atrophy. The ophthalmic featuresyndrome include epibulbar complex choristomaterior scleral cartilage. The management of com-oristoma depends upon the extent of the lesion.tion or wide local excision followed by mucousne graft reconstruction are options.

al conjunctival tumors

benign and malignant tumors can arise from theus epithelium of the conjunctiva.

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Papillom

Squamobe assoc16 andtumor clated thmotherpassesappearin a sesnumerostromachildrenin theshows nacantho

Several treatment options exist for small sessile papillo-mas in a child. Sometimes, periodic observation allows forslow spoLargersensatioincompprobablthe mas(no-toucthe viruappliedlesion iinstancethen exconjuncmycin Crecurrenrecurrenfollowinby boos

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ita

ditrareasiwalinabyoran rgn-byeem

354 Pediatrics and strabismus

Figure 4 Complex choristoma occupying the temporal half ofthe epibulbar surface, including the cornea, in a child withcutaneous nevus sebaceous of Jadassohn

Figure 5a

us papilloma is a benign tumor, documented toiated with human papillomavirus (subtypes 6, 11,18) infection of the conjunctiva [16,17]. This

an occur in both children and adults. It is specu-at the virus is acquired through transfer from thes vagina to the newborns conjunctiva as the childthrough the mothers birth canal. Papillomasas a pink fibrovascular frond of tissue arrangedsile or pedunculated configuration (Fig. 5). Theus fine vascular channels ramify through thebeneath the epithelial surface of the lesion. In, the lesion is usually small, multiple and locatedinferior fornix. Histopathologically, the lesionumerous vascularized papillary fronds lined by

sing

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HBIDt Lippincott Williams & Wilkins. Unauthorized

tic epithelium. Smallerocularsymptomwith mu

Squamo

Squamoneoplascells. Inwithin tdisplaysment minvasivemore eleither c

Patientstransplavirus or

Large conjunctival papilloma in an older childntaneous resolution of the viral-produced tumor.or more pedunculated lesions with foreign-bodyn, chronic mucous production, hemorrhagic tears,lete eyelid closure and poor cosmetic appearancey require surgical excision. Complete removal ofs without direction manipulation of the tumorh technique) is advisable to avoid spreading ofs [18,19]. Double freezethaw cryotherapy isto the remaining conjunctiva around the excisedn order to prevent tumor recurrence. In somes, the pedunculated tumor is frozen alone andcised while frozen or allowed to slough off thetival surface later. Topical interferon and mito-

have been employed for resistant or multiplyt conjunctival papillomas [2027]. For difficultt lesions, oral cimetidine for several monthsg surgical resection can minimize recurrenceting the patients immune system and suppres-virally stimulated mass [24].

ry benign intraepithelial dyskeratosis

ary benign intraepithelial dyskeratosis (HBID)benign condition seen in an inbred isolate of

an, African-American and American IndiansIndians). This group resided initially in North. It is an autosomal-dominant disorder character-bilateral elevated fleshy plaques on the nasal orl perilimbal conjunctiva and on the buccal mucosa.emain asymptomatic or can cause redness andbody sensation. It is characterized histopathologi-acanthosis, dyskeratosis on the epithelial surfacep within the epithelium, and prominent chronicatory cells.

does not usually require aggressive treatment., less symptomatic lesions can be treated withlubricants and topical corticosteroids. Larger

atic lesions can be managed by local resectioncous membrane grafting if necessary.

us cell carcinoma/conjunctival intraepithelial neoplasia

us cell carcinoma and conjunctival intraepithelialia (CIN) are malignancies of the surface epithelialtraepithelial neoplasia displays anaplastic cellshe epithelium whereas squamous cell carcinomaextension of anaplastic cells through the base-

embrane into the conjunctival stroma. Clinically,squamous cell carcinoma is usually larger and

evated than CIN. Leukoplakia can be seen withondition.

who are medically immunosuppressed for organntation, those with human immunodeficiencythose with underlying DNA repair abnormalitiesreproduction of this article is prohibited.

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like xeroderma pigmentosum are at particular risk todevelop conjunctival squamous cell carcinoma and malig-nant meing met

The mconjuncin the licornealvectomyponentremainior thosesive cormitomy

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Ocular mof the ppalate. Tconditioacquiredgraybrthe sclejunctivaaffectedimpartsmelanompatientsdevelop

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The contumor.secondslightly65% of cdarkly pcompletIt is typjunctivaout lifemalignabecomeevidenc

Histopaof nests

Conjunctival tumors in children Shields and Shields 355

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