conjunctival tumors

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Conjunctival tumors -Primary corneal tumors are exceedingly rare, and tumors effecting the cornea are usually extensions of conjunctival tumors. -Any conjunctival cell type can potentially lead to one or more particular conjunctival tumor(s). The majority of conjunctival tumors are benign. Malignant tumors of the conjunctiva are relatively rare. -conjunctival epithelial (including melanocytic) tumors are more common than conjunctival stromal tumors. -Background on the Conjunctiva -Main points: - at the limbus and lid margin the conjunctival epithelial cells are stratified squamous epithelium. (cuboidal at fornices and columnar on bulbar and tarsal surfaces) -The conjunctival stroma contains arteries, veins, nerves, fibroblasts, lymphoid cells (including histiocytes), primitive “myxoid” connective tissue, fat cells, lymphatic vessels, and other tissues. -embryologically conjunctiva is derived from surface ectoderm that is enclosed by the eyelid buds. The eyelid buds fuse and later separate, and sometimes leave eyelid tissue mixed in with the conjunctival tissue, especially on the bulbar surface (forming dermoids and sometimes eyelid colobomas). -Melanocytes (neural crest cells) are found in the basal cell layer of epidermis. They create melanin which is stored in melanosomes. Melanosomes are donated to keratinocytes and hair follicle cells. Melanin protects the skin from non-ionizing ultraviolet radiation. The number of melanocytes is the same among all races. The number and size of melanosomes are greater versus the distribution of melanosomes within keratinocyte cytoplasm is more widely spread in darker complexions. The number of melanocytes decreases with age and therefore complexions and hair lighten and the incidence of skin cancer increases. -Plica semilunaris is a vertical fold of conjunctival tissue lateral to caruncle -caruncle is a medial canthal fleshy prominence with cutaneous structures underlying mucosal structures. 1

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Page 1: Conjunctival tumors

Conjunctival tumors-Primary corneal tumors are exceedingly rare, and tumors effecting the cornea are usually extensions of conjunctival tumors.-Any conjunctival cell type can potentially lead to one or more particular conjunctival tumor(s). The majority of conjunctival tumors are benign. Malignant tumors of the conjunctiva are relatively rare. -conjunctival epithelial (including melanocytic) tumors are more common than conjunctival stromal tumors.-Background on the Conjunctiva

-Main points:- at the limbus and lid margin the conjunctival epithelial cells are stratified squamous epithelium. (cuboidal at fornices and columnar on bulbar and tarsal surfaces)-The conjunctival stroma contains arteries, veins, nerves, fibroblasts, lymphoid cells (including histiocytes), primitive “myxoid” connective tissue, fat cells, lymphatic vessels, and other tissues. -embryologically conjunctiva is derived from surface ectoderm that is enclosed by the eyelid buds. The eyelid buds fuse and later separate, and sometimes leave eyelid tissue mixed in with the conjunctival tissue, especially on the bulbar surface (forming dermoids and sometimes eyelid colobomas).-Melanocytes (neural crest cells) are found in the basal cell layer of epidermis. They create melanin which is stored in melanosomes. Melanosomes are donated to keratinocytes and hair follicle cells. Melanin protects the skin from non-ionizing ultraviolet radiation. The number of melanocytes is the same among all races. The number and size of melanosomes are greater versus the distribution of melanosomes within keratinocyte cytoplasm is more widely spread in darker complexions. The number of melanocytes decreases with age and therefore complexions and hair lighten and the incidence of skin cancer increases. -Plica semilunaris is a vertical fold of conjunctival tissue lateral to caruncle-caruncle is a medial canthal fleshy prominence with cutaneous structures underlying mucosal structures.

Where does cancer happen first?- carcinogenesis is a genetic disease

- Microbial agents: found within the host cell nucleus include viruses, but not bacteria or fungi, which may be why viral causes of conjunctival tumors outnumber other microbial causes. Chronic infectious processes of all types can cause chronic inflammation which can be carcinogenic. For this reason H. pylori has been determined to be a cause of cancer. Also, an association between Chlamydia and ocular lymphoma has been suggested. Human Papilloma Virus (HPV) (arguably according to some sources) and Kaposi sarcoma herpesvirus (definitely) are infectious causes of conjunctival tumors and are mentioned below.-Carcinogenic agents other than microbial, include: chemical, ultraviolet light and dietary

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-no chemical causes identified below. (Foreign bodies can cause lesions which simulate a tumor and they are mentioned below.)-ultraviolet light has been linked to ocular surface squamous neoplasia and skin cancers including melanoma. (“Ocular surface squamous neoplasia” has been coined as an umbrella term to include corneal and conjunctival involvement of conjunctival tumors.) In the genetic condition xeroderma pigmentosum the repair mechanism for DNA damaged by ultraviolet light does not function, increasing the rate of skin cancer 2000 fold. In another aside, the substitution of thymidine for cytosine in the tumor suppressor gene p53 is pathognomonic for ultraviolet light induced skin damage and is found in 90% of squamous cell carcinomas. Epidemiological studies determined that ultraviolet light exposure (and not HPV) is the main influence in the development of squamous cell carcinoma of the conjunctiva. -no dietary causes identified below. (Vitamin A deficiency and Bitot’s spots as a lesion simulating a tumor is mentioned below.)

- cancer: a disease of stem cells?-basal cells are stem cell-like in having proliferative capacity and a lack of differentiation. -The location of conjunctival stem cells is not known but they may be decentralized -Many of the lesions below occur at the limbus, which is believed to be a site of (corneal) stem cells.

Some NomenclatureThe name of a cancer is based on the proliferating cell type (and not the cell types which support the proliferating cells)

When epithelium is the proliferator of a malignancy then “carcinoma” is used

-squamous cell carcinoma means malignancy of squamous epithelium (as is found at the conjunctival limbus and lid margin)

When stroma is the proliferator of a malignancy then “sarcoma” is used

- lymphangiosarcoma means malignancy of lymphatic vessel (as is found in the conjunctival stroma)

The benign tumors of both epithelium and stroma have “-oma” endings.

-squamous papilloma means benign tumor of squamous epithelium

-lymphangioma means benign tumor of lymphatic vessel

Dysplasia – is mitosis occurring in a disordered fashion in suprabasalar epithelial cells. If the full thickness of epithelial cell layers are dysplastic it is known as “carcinoma in situ” or the closest thing to malignancy without being malignant. A malignancy would include breaking through the basement membrane and obtaining access to the circulation and thereby potentially causing metastasis.

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Acanthotic – means thickened epithelium

Leukoplakia – keratin formed on mucosal surfaces in white plaques

Major Types of Conjunctival TumorType SubtypesEpidermal Non-melanocytic MelanocyticStromal Vascular Fibrous Tissue

Neural HistiocyticMyxoid MyogenicLipomatous Lymphoproliferative

Congenital Hamartoma ChoristomaCaruncularMetastaticSecondarySimulating Lesions

Classification of Epidermal Tumors of the ConjunctivaType SubtypesNon-melanocytic Benign Squamous papilloma

Keratotic plaqueKeratocanthomaReactive Hyperplasia (pseudoepitheliomatous hyperplasia)Inverted Follicular keratosisHereditary intraepithelial dyskeratosisOncocytomaDacryoadenoma

Premalignant and malignant Actinic (solar) keratosisConjunctival intraepithelial neoplasiaSquamous cell carcinomaXeroderma pigmentosum

Melanocytic Benign Junctional nevusCompound nevusSpitz NevusBlue NevusPAM without atypiaCongenital melanosisRacial melanosis

Premalignant and malignant PAM with atypiaMelanoma arising from neviMelanoma arising in PAMMelanoma arising de novo

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Classification of Stromal Tumors of the ConjunctivaCategory SubtypesVascular Capillary hemangioma Cavernous hemangioma

Varix Racemose malformationHemangiopericytoma lymphangiectasiaKaposi’s Sarcoma lymphangiomaMalignant hemangioendothelioma

Fibrous Nodular fasciitis FibromaBenign fibrous histiocytoma Malignant fibrous histiocytoma

Neural Neurofibroma (localized) Neurofibroma (diffuse)Schwannoma (neurilemmoma) Granular cell tumor

Histiocytic Xanthoma Juvenile xanthogranulomareticulohistiocytoma

Myxoid myxomaMyogenic rhabdomyosarcomaLipomatous Lipoma Herniated orbital fat

liposarcomaLymphoproliferative Benign reactive lymphoid

hyperplasialymphoma

Leukemic infiltrates

Congenital tumors of the conjunctiva (hamartomas and choristomas)DermoidDermolipomaOsseous choristomaLacrimal gland choristomaComplex choristoma

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1. History – check old photographsa. If Since Childhood and….then consider…..

i. Deep (Scleral), immobile, Flat, Uniform pigment , can be patchy, Bilateral, bulbar, interpalpebral

1. congenital melanosisii. contains cysts ,Elevated, +/- pigment, suspicious if not Interpalpebral, feeder?,

well circumscribed, mobile1. nevus

iii. Limbal, Raised, White1. Dermoid (hairs) or other choristomas

b. If Acquired and… i. Pigmented and… then consider…

1. Variable pigmentation, flat, poorly circumscribed, mobilea. Primary Acquired Melanosis (PAM)

2. Nodular , var. pig., +/- surr. flat pigment, Feeder v., immobilea. Malignant Melanoma

ii. Nonpigmented and…1. Anywhere and…

a. Subepithelial, clear colorless fluidi. Epithelial cyst (not a tumor)

b. Solid and…i. Single or multiple, hx of systemic malignancy

1. Metastasisii. Reddish, velvety, hx of surgery or trauma

1. Pyogenic granuloma (not a tumor)2. Palpebral and…

a. Multiple, small, whitishi. Consider: sarcoid

b. Rock hard, waxy, brittlei. Amyloid

c. Salmon Pink , fleshy, solid, elevated, smooth, elderly pti. Lymphoma

3. Bulbar and…a. Bilateral, keratinized, shiny plaques

i. Family hx (autosomal dominant), “V” shape1. Benign intraepithelial dyskeratosis (Haliwa)

ii. Vitamin A deficiency1. Bitot’s spots (leukoplakia)

b. Older adult, yellowish, 3 and 9 oclock locations, fibrovascular (drags conjunctiva onto cornea), can be bilateral, wing shaped, smooth surfaced (rare keratosis), very slow growth, iron line, can be bilateral and nasal and temporal

i. Pinguecula/Pterygium

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c. Elderly pt , limbal, +/- adjacent corneal haze, unilateral,single (noninfectious), gelatinous, leukoplakic (minimal or absent in CIN), or papillary (strawberry-like)

i. Conjunctival intraepithelial neoplasia (CIN) (Also consider Keratotic plaque, Actinic keratosis, and Squamous cell carcinoma)

d. Youth , non-limbal , strawberry-like , can be bilat./multiple (infectious)

i. Squamous papilloma

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ii.

Classification of Epidermal Tumors of the Conjunctiva

Non-melanocytic Benign

1. Squamous papilloma

A solitary sessile squamous papilloma of the bulbar conjunctiva.

Two forms:

A. adult papilloma – LIMBAL; not infectious/not associated with HPV, so unilateral, solitary. sometimes may be difficult to distinguish from squamous cell carcinoma. Can be pigmented and simulate melanoma. Can develop in young adults who are immunosuppressed. One rare subtype has an inverted growth pattern involving underlying connective tissue. Tx with Excision.

B. childhood papilloma – NON-LIMBAL; HPV associated so can be multiple, bilateral. Singh: can be managed by observation alone. Usually there is spontaneous resolution. Shields: remove or freeze but avoid incomplete excision due to aggressive reoccurrence. Appears to have no malignant potential.

2. Keratotic plaque (and actinic (solar) keratosis which is listed below under premalignant)

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Keratotic plaque in bulbar conjunctiva posterior to the limbus in a 19-year-old man.Keratotic plaques are all leukoplakic. Impossible to differentiate from actinic keratosis. Difficult to differentiate benign from malignant. Only small chance of evolving into squamous cell carcinoma. Indistinguishable from CIN, which has slightly greater potential to convert to squamous cell carcinoma. Therefore the finding of leukoplakia in the conjunctiva is a relative indication for excision and cryotherapy, however it is acceptable to follow until progression is documented.

Actinic Keratosis develops slowly and often occurs within epithelium overlying existing pinguecula or pterygium. Believed to be due to prolonged ultraviolet exposure.

3. Keratocanthoma

Keratoacanthoma of bulbar conjunctiva temporally showing leukoplakia.

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Shields classifies keratoacanthoma as a form of reactive hyperplasia (pseudoepitheliomatous hyperplasia). This lesion is more inflammatory, with more rapid onset and more rapid progression, than its malignant counterpart squamous cell carcinoma.

Because Squamous Cell Carcinoma cannot be ruled out Tx- Excision

4. Reactive Hyperplasia (pseudoepitheliomatous hyperplasia)

Reactive Hyperplasia (Pseudoepitheliomatous hyperplasia) of the conjunctiva at the limbus inferotemporally. Note the leukoplakia secondary to hyperkeratosis.

Reactive hyperplasia is secondary to chronic inflammation often due to pterygium or pinguecula or at site of prior foreign body. This lesion is more inflammatory, with more rapid onset and more rapid progression, than its malignant counterpart squamous cell carcinoma.

Because Squamous Cell Carcinoma cannot be ruled out Tx- Excision

5. Inverted Follicular keratosis (no image)6. Hereditary intraepithelial dyskeratosis

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Associated with the Northeastern North Carolina Native American tribe, The Haliwa. Has also been found in individuals not of Haliwa ancestry.

Bilateral elevated fleshy plaques in a “V” shape. It has no known malignant potential. Basement membrane is intact. Tx – ocular lubricants, corticosteroids if needed.

7. Oncocytoma (caruncular was only image available)

Caruncular oncocytoma. Note the blue color and cystic appearance.

A Common lesion of lacrimal gland. Asymptomatic, slowly enlarging swellings in older individuals. Tx

Excision

8. Dacryoadenoma

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Rare, Tx Excision

Non-melanocytic Premalignant and malignant

9. Actinic (solar) keratosis (See Keratotic Plaque above)10. Conjunctival intraepithelial neoplasia (CIN)

Small gelatinous conjunctival intraepithelial neoplasia near limbus, simulating inflammation, in a 77-

year-old man.Can also be leukoplakic like keratotic plaque or papillomatous like squamous papilloma. (Beginning of a continuum, with the mildest form being squamous papilloma and the most extreme form of which transgresses the basement membrane and is called squamous cell carcinoma.) Predisposing factors include sunlight and HPV. Occurs frequently in immunosuppressed. Leukoplakia is absent or minimal in

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this particular lesion, but may be present in others. Extensive leukoplakia should raise suspicion of squamous cell carcinoma. Tx – Excision

The umbrella term OCULAR SURFACE SQUAMOUS NEOPLASIA (OSSN) includes dysplasia, CIN and squamous cell carcinoma of cornea and conjunctiva. OSSN: Use of 1% toluidine blue: One study of 47pts, presented earlier this year by Dr. Valle, showed that 90% of pts with premalignant and 100% of pts with malignant OSSN lesions showed positive staining.

11. Squamous cell carcinoma

Sessile papillomatous squamous cell carcinoma at nasal limbus in elderly patient. Note the slight

corneal invasion and the feeder vessels.Tx Excision. Occurs with much less frequency than CIN. Incidence 0.2-3.5 per 100,000. 75% in men and 75% in ages > 60yrs and >75% at limbus. Large dilated blood vessels frequently feed and drain the mass. Locally invasive but metastasizes in only 1-2%. Intraocular invasion may necessitate enucleation. Mucoepidermoid and spindle cell forms are less common but aggressive. Mucoepidermoid may originate in caruncle with a tendency toward orbital extension. Spindle cell form has a greater tendency to metastasize.

12. Xeroderma pigmentosum (no image) systemic condition with numerous lesions affecting sun exposed areas like the face.

Melanocytic Benign

13. Junctional nevus14. Compound nevus

Junctional and Compound nevi discussed together:

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Characteristic conjunctival nevus showing subtle clear cystic spaces.

Amelanotic nevus:

Light pink conjunctival nevus in a 13-year-old boy

Evolution of a nevus: Generally becomes clinically apparent in 1st or 2nd decade of life. The nevus begins as a small nest of cells in the basal layer (junctional) and in the 2nd to 3rd decade the cells migrate to underlying stroma (compound) and at this stage characteristic pseudocysts form. By the 3rd

or 4th decade the lesion has migrated entirely into the stroma (subepithelial). Usually no systemic

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associations, though rarely can be associated with Carney Complex (includes heart problems) and dysplastic nevus syndrome (high incidence of cutaneous melanoma).

Usually discrete, mobile lesion on bulbar surface. Can be deeply pigmented or amelanotic. Can become more pigmented with time.

-In contrast to melanoma, which is usually adherent to the globe, melanoses and nevi are movable with the conjunctiva.

Initial Mgt-periodic observation with photographs. If growth, consider excision, especially if family hx of melanoma.

15. Spitz Nevus (no image)16. Blue Nevus

Dark episcleral blue nevus in a 45-year-old woman.Blue nevus is deeper, may be attached to the sclera.

Initial Mgt-periodic observation with photographs. If growth, consider excision, especially if family hx of melanoma.

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17. PAM without atypia

Primary acquired melanosis in temporal conjunctiva in a 46-year-old woman

Mild PAM (less than 1 clock hour of conjunctival involvement)

Tx – Monitor for progression

18. Congenital melanosis (of the sclera)

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Scleral melanocytosis showing diffuse patchy brown pigment in superior aspect of the right eye.Though not conjunctival included here since in differential for PAM and conjunctival nevus.

No mgt necessary

19. Racial melanosis

Figure 19.61. Patchy complexion-related conjunctival pigmentation in right eye. Note the characteristic

linear spokelike deposits of pigment oriented perpendicular to limbus.No mgt necessary.

Melanocytic Premalignant and malignant

20. PAM with atypia

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Diffuse primary acquired melanosis inferiorly in a 48-year-old woman.

Moderate PAM with documented progression should be biopsied.

Relative indications for biopsy (Shields)

-lesion diameter >/= 5mm; documented progression, thickness of lesion, distinct nodule arising within lesion (pigmented or not), nutrient vessels to lesion, involvement of cornea, involvement of palpebral conjunctiva, dysplastic nevus syndrome in pt or close relative, personal hx of cutaneous or uveal melanoma, pt fear of cancer.

-Any nodule in PAM should be removed completely.

21. Melanoma arising from nevi

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Circumscribed melanoma at limbus, arising from a preexisting nevus in a 51-year-old woman. She

gave a history of a prior nevus at that site for many years, confirmed with inspection of prior

photographs.Tx Excision

22. Melanoma arising in PAM

Diffuse primary acquired melanosis in inferior fornix with forniceal and medial canthal melanoma.Tx Excision

23. Melanoma arising de novo

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Irregular melanoma near temporal limbus in a 45-year-old man.Tx Excision

Classification of Stromal Tumors of the Conjunctiva

Vascular

Pyogenic granuloma (not part of the classification scheme, but discussed here as part of vascular lesions)

Small pyogenic granuloma in inferior fornix.

Stains with Fluorescein.

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Tx with steroid early, or excision

24. Capillary hemangioma

Capillary hemangioma of the conjunctiva in a 6-month-old child. The lesion had grown progressively

for several weeks.

Mgt – observation since usually regresses. If amblyogenic tx with oral or intralesional steroid may

hasten resolution. If other tumors like rhabdomyosarcoma cannot be excluded exisional biopsy is

appropriate.

25. Cavernous hemangioma

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Cavernous hemangioma attached to the scleral surface. This teen-aged girl first noticed the lesion

while inserting a contact lens.

Can occur in associated with Sturge-Weber syndrome, blue rubber bleb nevus syndrome, and diffuse

neonatal hemangiomatosis.Mgt – periodic observation or local resection. Important to be sure not extension of orbital hemangioma.

26. Varix

Varix in superior bulbar conjunctiva in a 44-year-old woman. The lesion extended posteriorly into the

anterior portion of the orbit.Mgt – observe. if symptomatic or cosmetic, excision is possible with the understanding that the lesion can have posterior orbital component.

27. Racemose malformation (no image)28. Hemangiopericytoma

Hemangiopericytoma arising from the inferior fornix in a 40-year-old woman.

Neoplasm of vascular pericytes

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Tx – excision.

29. Lymphangiectasia

Conjunctival lymphangiectasia in a 24-year-old woman.May resemble cavernous hemangioma if filled with blood causing a blue-red or chocolate colored cyst. Usually represents a superficial component of deeper orbital lymphangioma.

Tx resection and cautery

30. Kaposi’s Sarcoma

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Conjunctival KS in an older patient who is presumably immunocompetent. He had a prior failed corneal

transplant in the contralateral right eye. There is a slight anterior bulging of left lower eyelid secondary

to the inferior conjunctival mass.

Can be the first sign of AIDS. Can resemble hemorrhagic conjunctivitis.

Mgt – chemotherapy and low dose radiation

31. Lymphangioma (also see Lymphagiectasia)

Hemorrhagic lymphangioma involving the nasal conjunctiva in the caruncle of a 40-year-old man.Tx resection and cautery

32. Malignant hemangioendothelioma (no image)

Other vascular lesions to be aware of:

Macrovessels

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Episcleral macrovessel located inferiorly in an otherwise normal eye. This vascular lesion is presumed

to be a congenital malformation and is not associated with ciliary body melanoma. However, detailed

examination to exclude a ciliary body melanoma is mandatory in such cases. Such a vessel can also be

seen with an iris arteriovenous communicaion.

Shown for comparison is an episcleral sentinel blood vessel overlying a ciliary body malignant

melanoma.

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33. Nodular fasciitis

Nodular fasciitis in epibulbar tissues superotemporally in an 11-year-old boy.Thought to originate from tenon’s capsule. Nodular, not encapsulated. Round or oval. Composed of fibroblasts. Can be misdiagnosed as sarcoma due to numerous mitotic figures.

Tx - Excision

34. Fibroma

Involvement of left conjunctiva with diffuse fibroma in a 74-year-old woman.

Rare. Slowly progressive stromal tumor acquired in adulthood. Composed of fibroblasts and collagen.

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Tx - Excision

35. Benign and malignant fibrous histiocytoma

Localized fibrous histiocytoma at limbus superonasally in an 8-year-old boy

Generally in adults, but a case of fibrous histiocytoma in a child with xeroderma pigmentosum has been reported. Amelanotic mass ranging from well-circumscribed to diffuse. Often at limbus. Mixture of spindle shaped fibroblasts and lipid-laden histiocytes formed from primitive mesenchyme. Malignant types are extremely rare can show marked pleomorphism, many mitotic figures, and multinucleated giant cells. Can metastasize to regional lymph nodes and hematogenously to distant organs causing death.

Tx- Excision if benign, radical surgery if malignant

Neural

36. Neurofibroma (localized) (Neuroma is included here as well)

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Circum scribed episcleral neurofibrom a in a 22-year-old woman. Although initially reported as being unassociated with neurofibrom atosis, the patient eventually developed NF-2

Peripheral nerve sheath (Schwann cell) tumor. Stromal solitary pink-yellow circumscribed or plexiform mass. Solitary type not usually associated with systemic disease, but plexiform type associated with neurofibromatosis type 1.

Tx – excise solitary lesion, debulk plexiform lesion

37. Neurofibroma (diffuse)

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Very subtle diffuse neurofibroma of the inferior bulbar conjunctiva in a young girl with NF-1.

Neurofibroma of eyelid

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Plexiform subconjunctival neuroma; Characteristic features of MEN-2B syndrome

38. Schwannoma (neurilemmoma)

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Epibulbar schwannoma in a 15-year-old boy. The lesion appeared and enlarged slowly over several

weeks.

Benign rare. Can arise in any part of conjunctiva. Pink-yellow elevated stromal mass. Schwann cell proliferation. Malignancy has not been reported.

Tx- Excision

39. Granular cell tumor

Granular cell tumor arising inferotemporally in the left eye of a 5-year-old girl.

Also known as myoblastoma. Very rare benign. Of disputed origin. Possibly Schwann cell. Pink elevated smooth well circumscribed stromal mass.

Tx - Excision

Histiocytic

40. Xanthoma (no image)

Yellow, subepithelial, epibulbar mass. In a case of xanthoma disseminatum, multiple lesions found including on limbus of both eyes. Infiltrate of Lipid-laden histiocytes and other cells.

41. Juvenile xanthogranuloma

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Conjunctival juvenile xanthogranuloma located at limbus in a 12-year-old girl

Solitary orange-pink stromal mass.

42. reticulohistiocytoma

Localized reticulohistiocytosis at the limbus in a 21-year-old woman.

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Histopathology of lesion showing large histiocytes with a granular cytoplasm

Myxoid

43. myxoma

Conjunctival myxoma of nasal conjunctiva in a 31-year old man.

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Myogenic

44. rhabdomyosarcoma

THE MOST COMMON MALIGNANT OCULAR TUMOR OF CHILDHOOD

Lipomatous

45. Lipoma

Conjunctival lipoma in a child with familial hypercholesterolemia.

46. Herniated orbital fat (no image)47. Liposarcoma (no image)

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Lymphoproliferative

48. Benign reactive lymphoid hyperplasia

Conjunctival benign reactive lymphoid hyperplasia presenting as a sessile lesion in the vicinity of the

medial rectus muscle in an 83-year-old woman.

49. Lymphoma

LEADING CAUSE OF CANCER DEATHS in <55yo group

Orbital lymphoma

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Conjunctival lymphom a presenting as a circum scribed mass near the limbus in a 43-year-old woman.

Diffuse lymphoma affecting medial aspect of conjunctiva in a 39-year-old woman

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50. Leukemic infiltrates

Diffuse conjunctival leukemic infiltrate in right eye of a 60-year-old man with chronic lymphocytic

leukemia.

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Congenital tumors of the conjunctiva (hamartomas and choristomas)

Fusion of lid folds (9th week of embryonic development)

51. Dermoid

Typical round conjunctiva at the corneoscleral lim bus inferotemporally.

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52. Dermolipoma

Sessile dermolipoma superotemporally in right eye of a 5-year-old girl

53. Conjunctival and corneal dermoids – embryological events

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Epibulbar dermoid and facial pit (lower left cheek) (and dermolipoma- not seen here) in Goldenhar syndrome

Preauricular skin appendages in the patient showing in Figure 16.4. The patient also had hearing loss,

consistent with Goldenhar syndrome.

54. Osseous choristoma

Typical epibulbar osseous choristoma in a 25-year-old woman.

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55. Lacrimal gland choristoma

Small lacrimal gland choristoma located near the limbus in a 40-year-old woman. The lesion had apparently been present since birth. It was excised because of a foreign body sensation. The diagnosis was not suspected clinically.

56. Complex choristoma

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Epibulbar complex choristoma in a child with the organoid nevus syndrome. The diffuse lesion covers

the lateral third of the cornea.

View of preauricular area showing tan sebaceous nevus

Caruncular

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Papilloma of caruncle in a 31-year-old woman.

Small noncystic caruncular nevus in a 40-year-old woman.

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Caruncular melanoma with involvement of the adjacent eyelid skin.

Lymphoma affected mainly the caruncle with some involvement of the adjacent conjunctiva in a 54-

year-old man.

Metastatic

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Lung cancer metastatic to the conjunctiva in a 55-year-old man. Subsequent systemic evaluation

revealed the primary lung cancer.

Breast cancer metastatic to the conjunctiva in a 55-year-old woman.

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Tan-colored mass in bulbar conjunctiva of a 48-year-old woman with a history of cutaneous melanom

a. She had no known metastasis at time of ocular presentation, but was subsequently found to have

widespread metastases.

Everted right upper eyelid showing multiple foci of metastatic melanoma on the tarsal conjunctiva

Secondary

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Sebaceous carcinoma of the caruncle in a 68-year-old woman. Note the clinical similarity to the sebaceous gland hyperplasia

Simulating Lesions

Inferotemporal epithelial inclusion cyst in bulbar conjunctiva in a middle-aged woman.

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Pigmented epithelial inclusion cyst simulating a conjunctival melanoma in a 62-year-old African

American patient.

Large, gray-black conjunctival lesion in a 24-year-old m an referred w ith the diagnosis of conjunctival m elanom a. History revealed that he had been hit in the eye with a pencil at age 7 years. The lesion had gradually enlarged over the last 5 years.

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Histopathology of the lesion showing inflam m atory reaction around lead foreign body

Bulbar conjunctiva of right eye of another patient. The lesion was removed and documented to be a

metallic foreign body.

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M ascara deposit (m ascarom a) near lim bus sim ulating conjunctiva melanoma in a patient who used

excessive mascara.

Diffuse episcleritis in a 67-year-old man. Acute-onset and Irritation.

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Nodular scleritis near the limbus in a 25-year-old woman. Gradual or acute. Painful.

Thickening and hyperemia of the upper tarsus in a 64- year-old man with Churg-Strauss syndrome.

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Ligneous conjunctivitis involving the palpebral and limbal conjunctiva in a 68-year-old woman. She had cataract surgery and pterygium about 5 months before the onset of these lesions.

Rare. Characteristic “woody,” thick membrane formation. Due to plasminogen deficiency, often autosomal recessive, may be sporadic.

Spontaneous staphylococcal abscess in a 47-year-old woman. The lesion extended through the sclera

necessitating a scleral graft. The patient had an excellent recovery after antibiotic treatment. Acute.

Painful.

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Solitary lesion of molluscum contagiosum at the inferior limbus in a patient with acquired immunodeficiency syndrome. Domed. May be chronic irritation.

Sarcoidosis was confirmed on conjunctival biopsy. The patient had systemic sarcoidosis but no other

ocular involvement.

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Serratia marscesens abscess simulating a conjunctival lymphoma. This yellow-pink lesion is in the bulbar conjunctiva of an elderly man who had prior cataract and glaucoma surgery. Acute

Conjunctival amyloidosis in inferior conjunctival fornix in a 50-year-old w om an, appearing as a subtle

pink thickening of the affected tissue. The first clinical diagnosis was lymphoma, but amyloidosis was a

secondary consideration.

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Slightly inflamed pinguecula nasally in a 51-year-old man.

Pterygium with severe secondary inflammation.

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Congenital uveal staphyloma in an 11-year-old boy.

Calcified scleral plaque in a 75-year-old woman

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Extraocular extension of uveal melanoma. Note the adjacent sentinel vessels

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