Conjunctival Disease: Tumors and Anatomic Abnormalities

Chapter 6
Conjunctival Disease: Tumors and

Anatomic Abnormalities
Conjunctival tumors can be divided into squamous neoplasms, melanocytic neoplasms, and
subepithelial neoplasms. It is often possible to differentiate benign lesions from malignant
lesions based on the appearance of the lesion; however, in some cases a biopsy is necessary.
Squamous Neoplasms of the Conjunctiva
Fig. 6.1 A sessile papilloma. This benign lesion of the conjunctiva consists of Fig. 6.2 A large papilloma. This large papilloma extends onto the corneal
multiple fibrovascular connective tissue cores with an overlying epithelium. Lesions surface.
may be sessile or pedunculated. They are caused by human papillomavirus
(subtypes 6, 11, 16 and 18), and in older adults, these lesions can be
premalignant.
Fig. 6.3 Condyloma acuminatum of the conjunctiva. Clinically, this

squamous papilloma extends from the caruncle deep into the inferior and superior
fornix of both eyes. In this case, molecular diagnostic polymerase chain reaction
studies confirmed human papilloma virus (HPV) subtype 11. Commonly, HPV 6
and 11 are benign strains associated with genital cervical warts. HPV 16, 18 and
31 can be associated with premalignant cervical lesions. When exuberant bilateral
squamous papillomas are derived from a sexually transmitted HPV infection, they
can be referred to as condyloma acuminatum of the conjunctiva.
44 Cornea Atlas
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Fig. 6.4 Condyloma acuminatum of the conjunctiva. Pathology

of the same case shown in Figure 6.3. The low-power specimen below
demonstrates a frond composed of a fibrovascular core (1) and
thickened epithelium (2). The higher magnification specimen above of
this HPV-induced squamous papilloma demonstrates binucleate cells (3)
and perinuclear cytoplasmic clearing (4), also known as koilocytosis.
Fig. 6.5 A conjunctival keratoacanthoma. This exceedingly rare lesion is

similar to a keratoacanthoma on the skin. The lesions arise rapidly, are elevated,
and contain a central keratin core.
Fig. 6.6 Conjunctival dacryoadenoma. Clinically, this is a soft, pink, mobile

mass. Histologically, it is composed of lacrimal secretory cells arising from the
conjunctival epithelium. It is a benign tumor. The lacrimal gland is embryologically
derived from the conjunctiva.
Conjunctival Disease: Tumors and Anatomic Abnormalities 45
Fig. 6.7 Hereditary benign
intraepithelial dyskeratosis. This
autosomal dominant condition is
primarily seen in the Haliwa Indians
(an inbred triracial isolate of white,
American Indian, and black origin)
of northeastern North Carolina. The
primary feature is white dyskeratotic
plaques on the peri-limbal bulbar
conjunctiva. Corneal and buccal
mucosal plaques have been described.
The lesions tend to wax and wane and
most cases can be treated with
conservative measures. Rarely, large
lesions require surgical resection.
Fig. 6.8 Conjunctival and corneal intraepithelial

neoplasia. This occurs predominantly in older Caucasians.
When it is seen in a younger patient, as in this case, a
thorough search for systemic immunodeficiency syndromes
should be undertaken. It usually begins at the limbus in the
interpalpebral region and often extends onto the cornea.
It is associated with chronic sun exposure. The lesions
are characteristically elevated and have a gelatinous
appearance. The conjunctiva is diffusely injected around
the lesion, and within the lesion, there are loops of vessels.
The lesion may resemble a papilloma.
46 Cornea Atlas
Fig. 6.9 Conjunctival intraepithelial neoplasia. In many cases, the lesion Fig. 6.10 Conjunctival and corneal intraepithelial neoplasia. The lesion
extends onto the cornea and has waxy, scalloped extensions (inset). in this case straddles the limbus and is more elevated.
Fig. 6.11 Corneal intraepithelial neoplasia. This is the appearance of the Fig. 6.12 Corneal intraepithelial neoplasia. The same patient as seen in
eye before initiating treatment with topical mitomycin 0.05%. Figure 6.11 six weeks after treatment with topical mitomycin 0.05%, the cornea
is less opacified. At that time, because of burning, the concentration was reduced
to 0.025% and used for 2 more weeks.
Fig. 6.13 Corneal intraepithelial neoplasia. The same patient as seen in Figure
6.11. This is the appearance of the eye seven months later. The visual acuity has improved
from hand motions to 20/200.
Fig. 6.14 Conjunctival intraepithelial neoplasia, pre-interferon
alpha-2b treatment. This 68-year-old male patient noted a lesion in his
right eye for several months. Impression cytology confirmed the diagnosis of
conjunctival intraepithelial neoplasia. The patient was started on topical interferon
alpha-2b four times a day.
Fig. 6.15 Conjunctival intraepithelial neoplasia. The same patient as seen

in Figure 6.14 two months after treatment with interferon alpha-2b. There is
partial resolution of the lesion.
Fig. 6.16 Conjunctival intraepithelial neoplasia. The same patient as seen

in Figure 6.14 three months after treatment with interferon alpha-2b. The lesion
has completely resolved.
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3
4 2
Fig. 6.18 Extensive squamous cell carcinoma of the conjunctiva. Initially

the patient refused resection and then returned with this advanced lesion.
5
Fig. 6.17 Conjunctival intraepithelial neoplasia. There is a junction

between normal epithelium on the right and neoplastic epithelium on the left (1).
Normal cells show a maturation continuum from immature cells in the basal layer
to mature anterior cells (2). In contrast, on the neoplastic side, immature cells
extend through the full-thickness of the epithelial layers (3). The basement
membrane underlying the neoplastic cells is intact (4). Beneath it are chronic
inflammatory cells (5).
Fig. 6.19 Extensive squamous cell carcinoma of the conjunctiva. The Fig. 6.20 Spindle cell carcinoma. Spindle cell carcinoma is a highly
same patient as in Figure 6.18. The lesion has metastasized to the preauricular malignant and aggressive tumor. It arises from the conjunctiva, limbus, or cornea
nodes circled. The nodes are firm, raised, and immobile. and may invade locally often resulting in severe ocular damage. Clinically, the
lesions are markedly white due to collagen deposition.
Melanocytic Neoplasms and Other Pigmented Lesions of
the Conjunctiva
Fig. 6.21 Nevi. These are benign pigmented lesions of the conjunctiva. They Fig. 6.22 Nevi. This is an example of a flat, pigmented conjunctival nevus.
usually occur on the bulbar conjunctiva and are freely mobile over the sclera. This Photography if properly done is the best way to follow pigmented lesions of the
is an elevated, lightly pigmented, conjunctival nevus. conjunctiva. They need to be photographed using a consistent technique that
includes repeated direction of gaze and a similar method of lighting.
Fig. 6.23 Nevi. Occasionally, they develop clear cystic spaces. Fig. 6.24 Nevi. Some are lightly pigmented or nonpigmented.
50 Cornea Atlas
Fig. 6.26 Malignant melanoma of the conjunctiva. This lesion can arise de
novo, from a pre-existing nevus, or from pre-existing PAM with atypia. This tumor
may appear on the palpebral conjunctiva or the bulbar conjunctiva and commonly
occurs at the limbus. It is elevated and highly vascular and is occasionally
accompanied by a hemorrhagic component, as in this case.
Fig. 6.25 Primary acquired melanosis (PAM). This is a flat, golden-brown

or tan pigmentation of the conjunctiva; its appearance may change with time.
This acquired lesion usually occurs in the fourth decade of life or later, primarily in
Caucasians. PAM with atypia may progress to malignant melanoma.
Fig. 6.28 Malignant melanoma of the conjunctiva and cornea. This is

another example of a conjunctival and corneal melanoma that is primarily located
on the cornea.
Fig. 6.27 Malignant melanoma of the conjunctiva and cornea. This is a

large conjunctival melanoma arising from the limbal region. The most common
sites of metastases are ipsilateral facial lymph nodes, brain, lung, and liver.
1
2
3
Fig. 6.29 Malignant melanoma of the conjunctiva and cornea.

Ultrasonography of the same patient as in Figure 6.28. Ultrasound biomicroscopy
shows the lesion (1) is superficial to Bowmans membrane (2) with normal thickness
of the underlying cornea (3).
1
2
Fig. 6.30 Histopathology of a conjunctival malignant melanoma. There
is invasion of the substantia propria with pleomorphic melanocytes (1) and
melanophages (2) with pigment.
Fig. 6.31 Melanosis. Pigmentation can occur around an Axenfelds intrascleral Fig. 6.32 Racial melanosis. This flat, deeply pigmented lesion of the
nerve loop. The pigmentation is found near the limbus and is benign and conjunctiva is commonly found near the limbus and in the interpalpebral area. It
nonmobile, since it is located within the sclera. These areas of pigmentation are develops early in life, is uninflamed, and rarely changes in appearance. It occurs
more common in African-American patients. primarily in darkly pigmented patients and has no malignant potential.
52 Cornea Atlas
Fig. 6.34 Paraneoplastic syndrome. This 45-year-old female presented with
a three month onset of multiple, well-circumscribed, bilateral bulbar and palpebral
conjunctival lesions. One week prior to presentation she was diagnosed with an
ovarian tumor. A rare paraneoplastic disorder, bilateral conjunctival pigment
proliferation can be the initial presenting sign of occult malignancy. This clinical
diagnosis is a variant of bilateral diffuse uveal melanocytic proliferation (BDUMP),
a recognized uveal pigment response to a primary gynecologic carcinoma.
Fig. 6.33 Striate melanokeratosis. This can be associated with pigmented

limbal lesions, such as racial melanosis. Chronic irritation of the corneal epithelium
results in migration of pigmented stem cells from the limbus onto the cornea. The
corneal lesion appears whorl-like, which represents the path of epithelial cell
migration.
Subepithelial Neoplasms and Other Lesions
Fig. 6.35 Lymphangiectasis. There is a collection of dilated lymphatic Fig. 6.36 Lymphangiectasis. A thin slit view shows the lymphatic channels to
channels within the conjunctiva. They are clear, elevated, and cystic. be cystic and elevated.
1
Fig. 6.37 Lymphangiectasis. Occasionally, this disorder has a hemorrhagic Fig. 6.38 Lymphangiectasis in Turners syndrome. Congenital
component. lymphedema is common in Turners syndrome. Lymphangiectasis of the
conjunctiva (1) may also be seen with this syndrome.
Fig. 6.39 Lymphangioma within the conjunctiva. These rare tumors Fig. 6.40 Lymphangioma within the conjunctiva. This is another example
usually occur during the first decade of life and often enlarge with upper of a lymphangioma within the conjunctiva. These lesions are characteristically
respiratory tract infections. In contrast to lymphangiectasis, these lesions are multiloculated and contain cystic channels.
composed of a proliferation of lymphatic channels and lymphoid tissue.
54 Cornea Atlas
Fig. 6.41 Lymphangioma involving lid. This patient had lid and orbital Fig. 6.42 Benign lymphoid hyperplasia. Lymphoid lesions of the conjunctiva
involvement. are typically elevated and salmon-colored. The surrounding tissue is uninflamed. A
local biopsy is necessary to establish the diagnosis.
Fig. 6.43 Subconjuctival lymphoma. This lesion is salmon-colored and has a

nodular or follicular appearance. All patients with biopsy proven subconjunctival
lymphoma need an extensive systemic work-up at the time of diagnosis and
frequently thereafter. The risk of developing systemic lymphoma is 1015% within
5 years of diagnosis and 28% within 10 years of diagnosis.
Fig. 6.44 Subconjunctival lymphoma. This is a sharply demarcated,

salmon-colored subconjunctival lymphoma. The mass is freely mobile and the
surrounding tissue in uninflamed.
Fig. 6.45 Conjunctival lesion in a patient with systemic lymphoma. Fig. 6.46 Kaposis sarcoma of the conjunctiva and lower lid in a
Similar to the lesions already noted, this is elevated and salmon-colored; the patient with AIDS. The lesion is elevated and highly vascular.
surrounding tissue is uninflamed.
Fig. 6.47 A limbal sarcoid nodule (inset).
Fig. 6.48 Multiple sarcoid nodules in the inferior fornix. These solid,
raised lesions (inset) may resemble follicles.
56 Cornea Atlas
Fig. 6.49 Hemangiomas. These lesions rarely occur in the conjunctiva. Fig. 6.50 Conjunctival hemangiomas in a patient with Osler-Weber-
Rendu disease.
Fig. 6.51 A pyogenic granuloma. This lesion arises after inflammation of the Fig. 6.52 A suture granuloma after strabismus surgery.
conjunctiva and is often seen after a surgical procedure. It is composed of
granulation tissue, fibroblasts, and capillaries and does not contain granulomas.
Fig. 6.54 Nodular fasciitis. This lesion is an acute proliferation of sheets of
immature fibroblasts. It occurred in response to multiple self-inflicted needle
punctures to the conjunctiva.
Fig. 6.53 A recurrent keloid on the left cornea in a patient with Lowe
syndrome. Unlike a pterygium, this lesion extends from the inferior limbus onto
the cornea and deep into the corneal stroma.
Fig. 6.55 Primary localized amyloidosis. In primary localized amyloidosis Fig. 6.56 Primary localized amyloidosis. This is the left eye of the same
there is deposition of abnormal extracellular protein, usually composed of patient seen in Figure 9.67. Amyloid deposits are seen in the palpebral conjunctiva
immunoglobulin light chains. The protein is identified histologically by Congo red of the left eye. Primary localized amyloidosis is a diagnosis of exclusion. Patients
staining. When examined with a polarized light, the characteristic apple-green must be worked-up for signs of systemic amyloidosis (see Figure 9.68).
birefringence pattern is seen. This patient has amyloid infiltration in the caruncle
region of the right eye.
58 Cornea Atlas
Fig. 6.57 Primary localized amyloidosis. Congo red stain reveals amyloid Fig. 6.58 Primary localized amyloidosis. Birefringence of the Congo red
deposits. stain is seen with polarized light.
Fig. 6.59 A xanthogranuloma on the cornea of an 8-year-old boy. Fig. 6.60 A presumed xanthogranuloma in a 9-year-old boy.
Xanthogranulomas can also occur on the skin (see Figure 3.2) and the iris.
Children with iris xanthogranulomas may initially present to the ophthalmologist
with visual problems related to spontaneous hyphemas.
Fig. 6.61 Conjunctival ectopic lacrimal gland tumor. This bilobed, fleshy,
choristoma tumor arose from ectopic lacrimal tissue in the conjunctiva. The
lacrimal gland is embryologically derived from the conjunctiva. This lesion extends
deep into the sclera and cornea making complete resection difficult.
Fig. 6.62 Conjunctival cyst. A conjunctival cyst is seen in direct illumination.
Fig. 6.63 Conjunctival cyst. The same lesion as seen in Figure 6.62 in indirect
illumination. Inclusions are present within the cyst.
Fig. 6.64 Large conjunctival cyst arising from the palpebral

conjunctiva. The central cyst is clear, and the margins of the cyst are easily seen.
60 Cornea Atlas
Prolapsed Orbital fat
Fig. 6.65 Prolapsed orbital fat mistaken for a

conjunctival cyst. In contrast to the conjunctival cyst in
Figure 6.64, this lesion is not clear and the margins cannot
be easily seen. With digital pressure, the prolapsed fat can
be pushed back into the orbit, but it returns when the
pressure is removed.
Conjunctivochalasis
Fig. 6.66 Conjunctivochalasis. Conjunctivochalasis is a redundancy of the Fig. 6.67 Conjunctivochalasis. This is the same patient as seen in Figure 6.66.
conjunctiva, usually overhanging the lower lid (1). Common symptoms include There is pooling of fluorescein dye (1) where the conjunctiva overhangs the
irritation, tearing and redness. Most cases require no treatment. Medical treatment lower lid.
includes topical lubricants, topical antihistamines and topical corticosteroids.
Conjunctival resection is performed in patients with severe symptoms who have
failed medical treatment.

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Chapter 6

Conjunctival Disease: Tumors and

Squamous Neoplasms of the Conjunctiva

Fig. 6.3 Condyloma acuminatum of the conjunctiva. Clinically, this

Fig. 6.4 Condyloma acuminatum of the conjunctiva. Pathology

Fig. 6.5 A conjunctival keratoacanthoma. This exceedingly rare lesion is

Fig. 6.6 Conjunctival dacryoadenoma. Clinically, this is a soft, pink, mobile

Conjunctival Disease: Tumors and Anatomic Abnormalities 45

Fig. 6.8 Conjunctival and corneal intraepithelial

Conjunctival Disease: Tumors and Anatomic Abnormalities 47

Fig. 6.15 Conjunctival intraepithelial neoplasia. The same patient as seen

Fig. 6.16 Conjunctival intraepithelial neoplasia. The same patient as seen

Fig. 6.18 Extensive squamous cell carcinoma of the conjunctiva. Initially

Fig. 6.17 Conjunctival intraepithelial neoplasia. There is a junction

Conjunctival Disease: Tumors and Anatomic Abnormalities 49

Fig. 6.25 Primary acquired melanosis (PAM). This is a flat, golden-brown

Fig. 6.28 Malignant melanoma of the conjunctiva and cornea. This is

Fig. 6.27 Malignant melanoma of the conjunctiva and cornea. This is a

Fig. 6.29 Malignant melanoma of the conjunctiva and cornea.

Conjunctival Disease: Tumors and Anatomic Abnormalities 51

Fig. 6.33 Striate melanokeratosis. This can be associated with pigmented

Subepithelial Neoplasms and Other Lesions

Conjunctival Disease: Tumors and Anatomic Abnormalities 53

Fig. 6.43 Subconjuctival lymphoma. This lesion is salmon-colored and has a

Fig. 6.44 Subconjunctival lymphoma. This is a sharply demarcated,

Conjunctival Disease: Tumors and Anatomic Abnormalities 55

Fig. 6.47 A limbal sarcoid nodule (inset).

Conjunctival Disease: Tumors and Anatomic Abnormalities 57

Conjunctival Disease: Tumors and Anatomic Abnormalities 59

Fig. 6.64 Large conjunctival cyst arising from the palpebral

Fig. 6.65 Prolapsed orbital fat mistaken for a

Conjunctival Disease: Tumors and Anatomic Abnormalities 61

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