Benign and Malignant Tumors of the Conjunctiva

review of missed concepts on exam 2

conjunctival general anatomy

1. clear mucus membrane

2. over sclera = bulbar

3. eyelids = palpebral

4. redundant area at corner = fornix

conjunctival anatomy

1. tear film

2. conj epithelium

   1. basal —> squamous

   2. basal = melanocytes

   3. goblet cells = produce mucin

      1. helps tear film adhere to opical surface

3. basement membrane

   1. barrier

4. lamina propria

   1. adenoid layer

      1. highly lymphatic

         1. has lymphocytes (inflammatory cells)

   2. blood supply

   3. fibrous later

      1. connective tissue

      2. tensile strength

tenons capsule — not part of conj

sclera

what is special ab squamous ep of conj

NON keratinized

what is a choristoma

Tumor-like growth composed of normal tissue in an abnormal location

what are the 2 choristomas

1. dermoid

2. dermolipoma

Conjunctival Dermoid cause

Benign congenital choristomas of conjunctiva

epi of conjunctival dermoid

1. random

2. clinically present in YA

3. can be associated w Goldenhar syndrome

   1. eye, ear, spine

pathophys of conjunctival dermoid

1. “Sequestration” (separation of tissue) of conjunctival epithelium during development.

2. • Simple: One tissue type

3. • Complex: >1 tissue type

4. • Tissue can consist of pilosebaceous unit (hair), sweat glands, fat, skin, and connective tissue.

clinical presentation of conj dermoid

1. Variable size, yellow -white limbal mass often found Nasal quadrant

2. • May contain fine hairs .

3. • May have extensive corneal involvement causing irritation, astigmatism, and incomplete eyelid closure.

how do you treat conj dermoid

1. removal - if inducing astig, amblyopia, reducing vision, cosmesis

Conjunctival Dermolipoma epi

1st or 2nd decade of life

pathophys of conj dermolipoma

Consist of adipose (Fat) tissue and is contiguous with conjunctival epithelium Can also be associated with Goldenhar Syndrome

clinical presentation

• Less defined than dermoid, pale -white, soft “fatty” lesions fixed to the conjunctiva • Can have fine hairs • Location: Often at superotemporal fornix

what are the beningn ep tumors involving

conj ep

what are the 3 benign epithelial tumors

conj papilloma cause

like veracuca vulgaris

from HPV 6 and 11

pathophys of conj papilloma

1. Human Papilloma Virus is tumorigenic and promote squamous cell proliferation of conjunctival epithelium —> superficial

2. • Most often benign and low risk for malignancy potential

childhood form of conj papilloma appearance

1. • Single or multiple

2. • Sessile or pedunculated appearance

3. • Fleshy red appearance due to many fine vascular channels and has a vascular core

4. • Location: Inferior fornix, bulbar conjunctiva, rarely touching cornea

adult form of conj papilloma

1. resembles Conjunctival Intraepithelial neoplasia (CIN)

   1. cant tell the difference

2. single and isolated

3. near limbus and can encroach or cover cornea

management of conj papilloma

excision

Hereditary Benign Intraepithelial Dyskeratosis(HBID) cause

Benign inherited disease of the conjunctival and oral mucosa

epi of Hereditary Benign Intraepithelial Dyskeratosis(HBID)

1. • Rare

2. • Autosomal dominance (AD) pattern of inheritance

3. • Native American heritage: Haliwa-Saponi tribe ancestry

   1. • Predominantly in North Carolina region

4. • Onset is early childhood with periods of wax and waning

pathophys of Hereditary Benign Intraepithelial Dyskeratosis(HBID)

Exact unknown, abnormally thickened squamous epithelium and keratinization of conjunctiva and oral mucosa.

so interferes w tear film

Hereditary Benign Intraepithelial Dyskeratosis(HBID) appearance

1. • Bilateral conjunctival injection (redness)

   1. • Dilated conjunctival vessels giving red eye appearance

2. • Bilateral white -gray, painless, elevated gelatinous, spongy, plaques

   1. • Location: nasal or temporal perilimbal area with possible extension onto cornea

   2. • Also apparent on oral cavity • Mouth, lip, tongue

management of HBID

treat ocular surface (DED) from it

can excise plaques if it affects it — they come back

Conjunctival Epithelial Inclusion Cyst cause

1. • Primary form is congenital

2. • Secondary form is spontaneous or due to inflammation, trauma or surgery

pathophys of Conjunctival Epithelial Inclusion Cyst

Inclusion of conjunctival epithelium into the substantia propria (stromal region) forming a cystic cavity filled with shed cells or mucoid material

appearance of Conjunctival Epithelial Inclusion Cyst

1. • Smooth, thin-walled cyst filled with clear serous fluid

2. • Epithelial debris settles on the bottom forming “pseudohypopyon”

whats the 3 benign melanocytic tumors

1. melanocytic nevus

2. ocular melanocytosis

3. complxion associated melanosis (CAM)

Conjunctival Melanocytic Nevus cause

Congenital lesions consisting of nests of melanocytes

epi of Conjunctival Melanocytic Nevus

1. • Caucasians> Blacks>Asians, Hispanics

2. • Malignant transformation <1%

3. • Onset since birth/childhood - congenital

4. • Most common pigmented conjunctival tumor

Conjunctival Melanocytic Nevus pathophys

Infiltration of distinct nest of benign melanocytes near the basal layer of conjunctival epithelium and migrates into underlying stroma with age

3 stages

1. junctional

2. compound

3. subethileal

Conjunctival Melanocytic Nevus appearance

1. solitary

2. range of pigmentation

   1. flesh to dark

3. interpalpebral bulbar conj

4. junctional

   1. younger age

   2. flatter

   3. less pigment

   4. at ep

5. compound

   1. adults

   2. development of cysts

   3. at level of stroma and protruding in ep

6. subepithelial

   1. older adults

   2. less pigmented

   3. pseudocyts

   4. level of stroma entirely wo ep component

assessing conj melanocytic nevus

1. OCT - Anterior Segment

   1. melanocytic nevus —> thickened epithelium (hyporeflective band)

   2. often have cysts!!

ocular melanocytosis — nevus of ota cause

Congenital pigmentation of periocular skin, uveal tract, sclera sometimes orbits, meninges, and palate.

IF skin involved → Nevus of Ota

epi of ocular melanocytosis

• Present at birth or during 1st year of life •

More common in Asians and blacks, Females > Males

• HOWEVER, Higher risk of malignant uveal melanomas in whites 1:400

clinical presentation of ocular melanocytosis

• Flat, blue-grayish, patchy pigmentation of conjunctiva and sclera; Tends to follow CN 5 V1 & V2 distribution • Can have iris mammillations • Often Unilateral, bilateral in only10% cases • Risk of congenital glaucoma

Complexion Associated Melanosis (CAM) aka

racial melanosis

cuase of CAM

Also known as racial melanosis, benign melanocytic lesion found among darkly pigmented individuals present since childhood or birth

pathophys of Complexion Associated Melanosis (CAM)

Hyperpigmentation of the basal epithelium without melanocytic proliferation, melanocytes are contained to level of basal epithelium without hyperplasia

CAM presentation

1. • Typically bilateral

2. • Flat, non-cystic pigmentation that can cover the conjunctiva extensively and increase in size with age

3. • Ill-defined margins

4. • May have spoke-like appearance

5. • Location: around corneoscleral limbus

what are the benign vascular/lymphatic tumors (3)

Lymphangiectasia/ Lymphangioma cause

1. • Congenital form related to developmental disorders.

2. • Acquired form may be related to trauma, inflammation, or radiation.

Lymphangiectasia/ Lymphangioma pathophys

1. • Dilated and prominent lymphatic channels of conjunctiva → Lymphangiectasia — string of pearls — looks like mucus on conj

2. • When it becomes mass-like and diffuse →Lymphangioma

3. • Filled with lymph fluid (clear)

Lymphangiectasia/ Lymphangioma presentation

1. • Diffuse distribution of lymph with chemosis

2. • Localized appearance, "string of pearls”

3. • Bleeding into lymph channels cause hemorrhagic lymphangiectasia

Conjunctival Varix cause

Venous malformation that is typically an extension of orbital varix, often rarer to be isolated to conjunctiva only

Conjunctival Varix pathophys

One or more dilated venous channels caused by vascular dysgenesis leading to thrombosis

Conjunctival Varix appearance

1. • Large distinct blood vessels

2. • May have faint blue-black color

3. • Freely mobile and not fixed to the sclera

managing Conjunctival Varix

1. • Isolated conjunctival varix is asymptomatic but may be excised for cosmetic considerations •

2. Consider orbital varix with proptosis, pain, optic nerve compression.

Conjunctival Capillary Hemangioma congenital pathophys

Vascular endothelial cell proliferation filled with blood

clinical presentation of congenital Conjunctival Capillary Hemangioma

• Solitary, distinct, red mass, variable size • Unilateral • May progressively enlarge for up to 2 years then gradually spontaneously regress

acquired capillary hemangioma clinical presentation

1. • Sessile/immobile, flat lesion with convoluted intertwining blood vessels

2. • Often smaller in size

3. • Often remain stable in size without growth

what are other benign lesions

pingeucula pathophys

Related to excessive exposure to UV light, dust, wind and other harsh elements

Zone of thickened conjunctival stroma replaced by degenerated collagen and abnormal elastic tissue

pterygium cause

Related to excessive exposure to UV light, dust, wind and other harsh elements

pterygium clinical presentation

1. • Dragging of conjunctival tissue and blood vessels across the cornea •

2. Triangular appearance •

3. Location: Pinguecula extending onto superficial cornea, nasal and temporal

Bitot’s spots cause

Belongs to a range of ocular manifestations called xerophthalmia because of Vitamin A Deficiency (VAD)

epi of bitots spots

1. • In developing countries •

   1. Malnourished individuals •

   2. Pregnant and nursing mothers

2. • In developed countries

   1. • Patient with GI disorder = malabsorption •

   2. Patients with liver, bowel, pancreatic disease = impaired Vit A storage

   3. • Patients on strict vegetarian or vegan diets

pathophys of bitots spots

1, • Vitamin A required for normal epithelium

• VAD → Abnormal conjunctival epithelium keratinization

bitots spot appearance

1. • White-Grayish triangular patches of xerosed (dried) conjunctiva with foamy surface •

   1. The tear film does not “wet” the surface •

2. Location: Temporal conjunctiva, Often bilateral

concretions pathophys

Degenerated epithelial cells and proteins become trapped in subconjunctival space and become calcified

management of concretions

• Remove with forceps or needle if eroding through epithelium and causing foreign body sensation

what are the premalignant lesions

primary acquired melanosis (PAM) epi

• Found in lighter pigmented individuals

• 50% progress to melanoma

• Onset is middle aged and older

pathophys of PAM

Abnormal melanocytes in the basal layers of epithelium

PAM appearance

1. • Almost always unilateral

2. • Flat, diffuse, ill-defined borders

3. • Varies in pigment from light to dark brown

4. • Location: limbus and bulbar conjunctiva, can extend to palpebral conjunctiva, caruncle, cornea and eyelid = more suspicious

CAM vs PAM

1. CAM

   1. darker complexion

   2. since childhood or birth

   3. bilatral around limbus

   4. no melanocyte proliferation

   5. observe

2. PAM

   1. lighter complexion

   2. since middle aged or older

   3. unilateral and can extend to palpebral conj, caruncle, cornea

   4. abnormal melanocyte proliferation

   5. treat if >2 clock hours —> biopsy for concern of melanoma

Conjunctival Intraepithelial Neoplasia (CIN) cause

Acquired precancerous lesion arising from various environmental and genetic risk factors

epi/risk factors of CIN

1. • Fair skinned individuals

2. • Middle-aged or older individuals

3. • History of chronic UV exposure •

4. Immunosuppressed individuals such as those with HIV

5. • Tobacco use, exposure to petroleum products

6. • Infection with Human papilloma virus (HPV 16, 18)

pathophys of CIN

Slow growing, non-invasive, localized tumor with thickened, mutated, and irregular epithelial cell arising from limbal stem cells; however, basement membrane is intact and underlying substantia propria is spared

• Precursor to squamous cell carcinoma

CIN belongs to what disease

OSSN

ocular surface corneal intraepithelial neoplasia

papilliform of CIN appearance

• Fleshy, papillomatous appearance

gelatinous from of CIN appearance

• Hairpin configuration of vessels around a sessile/flat lesion

leukoplakic form of CIN appearance

• Whitened and thickened surface with surface hyper -keratinization

what is true of all CIN appearance

Typically mobile, can have feeder vessels

• Location:

• Common: Limbus and interpalpebral bulbar conjunctiva (nasal & temporal)

• Less common: palpebral conjunctiva and fornix

additional assesment of CIN

1. OCT ant seg

   1. high resoln!!!

   2. Distinctive, thickened hyperreflective epithelial layer with an abrupt transition from normal to abnormal epithelium

      1. NO CYSTIC POCKETS

2. rose bengal or lissamine green vital dye

   1. (+) staining/ stipping

   2. stain dying cells

   3. can delineate extent of CIN but non specific

managing CIN

1. Complete excision recommended

2. • Surgical •

3. Chemotherapeutics

what are the malignant conj tumors

Conjunctival Invasive Squamous Cell Carcinoma cause/pathophys

1. CIN is precursor

2. Acquired cancerous lesion arising from precursor lesions such as Conjunctival Intraepithelial Neoplasia (CIN).

3. a (CIN) breaches the basement membrane of epithelium and invades underlying stroma and other structures

clinical presentation of Conjunctival Invasive Squamous Cell Carcinoma

1. Similar to Conjunctival Intraepithelial Neoplasia (CIN), difficult to clinically differentiate between the two

   1. • Can have all characteristics of CIN:

      1. • Papillomatous, Gelatinous, Lekoplakic

   2. • Can have: • (+) large, dilated feeder vessels

location of Conjunctival Invasive Squamous Cell Carcinoma

Limbus and interpalpebral bulbar conjunctival (nasal & temporal) and can invade into cornea, orbit and globe —> invasive

Conjunctival Malignant Melanoma cause

1. Acquired cancerous melanocytic lesion that either arise from:

   1. Primary acquired melanosis (PAM)* *Most common

   2. Conjunctival nevus

   3. . De novo (no pre-existing lesion)

pathophys of Conjunctival Malignant Melanoma

Malignant melanocytes that invade the conjunctival stroma and have access to lymphatic channels for metastasis → brain, liver, skin and bone

Conjunctival Malignant Melanoma presentation

1. • Pigmented, elevated lesion with illdefined margins

2. • Can range in pigment: black to flesh colored (amelanotic)

3. • May have feeder vessels

4. • Can range in size

5. • Location:

   1. • More common: bulbar conjunctiva near

   2. • Less common: fornix or palpebral conjunctiva

pathophys of Conjunctival Malignant Lymphoma

1. Lymphoma is monoclonal proliferation of B and T lymphocytes

   1. • Uncontrolled replication of single mutated lymphocyte

2. • Most common subtype of conjunctival lymphoma is:

   1. • Non-Hodgkin, Extra-nodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-type lymphoma)

conjunctival malignant lymphoma clinical presentation

1. • Diffuse, slightly elevated, fleshy pink mass

   1. • Salmon patch

   2. • “Chemotic” appearance

      1. • Mimics inflammatory conjunctivitis

2. • Most often smooth but follicular subtype has multinodular appearance

3. • Location:

   1. • More common: Fornix, superior and inferior bulbar conjunctiva

   2. • Less common: Limbus

how else can we assess conjunctival malingnant lymphoma

OCT (high resoln)

• hyporefelctive/dark

• smoooth subepithelial lesion

conj Kaposi sarcoma review

1. HPV 8 - cause

2. AIDS/ renal transplant

3. males

4. Network of proliferating malignant vascular endothelial cells with blood filled spaces

5. single - multiple red, purple, brown, blue lesions

6. locations wi ocualr structures

   1. • Eyelid, conjunctiva (bulbar, palpebral, caruncle), lacrimal sac