SURVEY OF OPHTHALMOLOGY VOLUME 48 • NUMBER 6 • NOVEMBER–DECEMBER 2003

MAJOR REVIEW

Anterior Segment Tumors: Current Concepts

and Innovations
Flavio A. Marigo, MD1,2, and Paul T. Finger, MD1,3,4

1
The New York Eye Cancer Center; 2The Federal University of Minas Gerais and Instituto da Visão, Belo Horizonte,
Brazil; 3The New York Eye and Ear Infirmary; and the 4New York University School of Medicine, New York, New York, USA

Abstract. The most common anterior segment tumors are primary neuroepithelial cysts, uveal
melanomas, metastatic tumors, and benign tumors. In the majority of cases, the diagnosis can be made
utilizing a careful clinical history and ocular examination. Ultrasound examination (low and high
frequency) has become an indispensable tool used to determine tumor extension and involvement of
the surrounding structures. In particular, high-frequency ultrasound has been used to uncover iris
pigment epithelial cysts, to allow for the diagnosis of small ciliary body melanomas, and to measure
tumors for plaque radiation planning. Whereas fluorescein angiography and computerized tomography
have come to play a limited role, fine-needle aspiration biopsy has been found to be quite helpful in
selected cases. Once the diagnosis is established, treatment decisions depend on the tumors’ location, size,
local extension, patterns of growth, and secondary complications. Most anterior segment tumors can
be observed for growth prior to treatment. Other options include local resection (iridectomy, lamellar
sclerouvectomy, or eye-wall resection) and radiation (ophthalmic plaque or external beam). Enucleation
is typically employed if these eye- and vision-sparing treatments are not possible and for uncontrollable
secondary glaucoma. This review examines the unique role of high-frequency ultrasonography for
the diagnosis and treatment of anterior segment tumors as well as an overview of clinical practice.
(Surv Ophthalmol 48:569–593, 2003. 쑖 2003 Elsevier Inc. All rights reserved.)

Key words. cancer • ciliary • cysts • glaucoma • iris • melanoma • metastasis •

nevus • retinoblastoma • tumors

Anterior segment tumors typically originate from the
iris and ciliary body and rarely from the cornea or
lens. The majority of acquired anterior segment neo-
plasms are benign iridociliary cysts, which are fre-
quently undetected during routine ophthalmic
examination. However, anterior segment cysts can
enlarge enough to cause compression and disloca-
tion of the iris and ciliary processes.
Malignant melanoma is the most frequent primary
malignancy in the anterior segment. Compared to
posterior uveal melanomas, iris melanomas tend to be
smaller and visible, so they are detected early and
rarely metastasize.40,44,118,136,148–150,157,190,250 In con-
trast, ciliary body melanomas are considered more
malignant.112,190,246 Hidden behind the iris, ciliary
body melanomas are usually detected after they have
become relatively large.139,246
Treatments of anterior segment tumors include
local resection (e.g., iridectomy, sector iridectomy,
lamellar sclerouvectomy, and full-thickness eye-
wall resection)60,110,215,216 and ophthalmic radiation
therapy.84,86,248,284 Enucleation is typically employed
if all else fails and for uncontrollable secondary
glaucoma.200

569
쑖 2003 by Elsevier Inc. 0039-6257/03/$–see front matter
All rights reserved. doi:10.1016/j.survophthal.2003.08.001
570 Surv Ophthalmol 48 (6) November–December 2003
This review reveals how trans-corneal fine needle
aspiration biopsy has become more important and
that high-frequency ultrasound has become indis-
pensable for diagnosis, surgical planning, and radia-
tion treatment of anterior segment tumors.
I. Pertinent Anatomy and Histology
of the Anterior Uvea
Most of the primary anterior segment tumors arise
from cells of the anterior uvea. For practical pur-
poses, the anterior uvea is composed by the iris and
the ciliary body.
A. IRIS
Histologically, the iris is divided into three layers:
• The anterior border layer, which is a discontinu-
ous, although relatively compact layer of fibroblasts
and melanocytes.
• The iris stroma, which has a less compact arrange-
ment with a relative paucity of cells (mainly fibro-
blasts and melanocytes and some mast cells). Most
of the nerve fibers and blood vessels are found in
the stroma as well as the sphincter and dilator
muscle fibers.
• The posterior layer, which is the iris pigment epi-
thelium, is composed of two layers of epithelial
cells.
Embryologically, the iris pigment epithelium, the
sphincter muscle, and the dilator muscle are of neu-
roectodermal origin whereas the other iris structures
and cells are of mesectodermal origin.106
B. CILIARY BODY
Histologically, the ciliary body is composed of the
following layers: (a) the ciliary epithelium, (b) the
stroma and muscle, (c) the supraciliary space. The cil-
iary epithelium is constituted of an inner nonpig-
mented layer and an outer pigmented layer. The
stroma is composed of collagen layers, of ciliary mus-
cles and of cells (fibroblasts, melanocytes, scattered
mast cells, vessels, and nerves). The supraciliary space
is a delicate collagen layer that separates the ciliary
body from the sclera. Axenfeld loops (of nerves and
blood vessels) extend from the ciliary body through
the sclera. They are typically located in the inferior
quadrants and appear as a dark spot under the con-
junctiva. Axenfeld loops can provide a route for a
direct extension of neoplastic cells from the ciliary
body through the eye wall (Fig. 1). Embryologi-
cally, both the nonpigmented and the pigmented
ciliary body epithelium are of neuroectodermal
origin while the other structures, including the ciliary
body muscle, are of mesectodermal origin.105
MARIGO AND FINGER
Fig. 1. Pigmented perivascular deposits are noted to sur-
round a dilated emissary blood vessel in a case of ciliary
body melanoma.
II. Clinical Evaluation of Anterior
Segment Tumors
The basic work-up for anterior segment tumors
involves a complete ophthalmic examination, which
typically includes a past medical history, best
corrected visual acuity, tonometry, slit-lamp biomi-
croscopy, gonioscopy, ophthalmoscopy, and transillu-
mination. High-resolution photographs or a drawing
of the tumor should be performed to document its
visible characteristics. Ancillary examinations may in-
clude ultrasound examination (conventional and
water-bath ultrasonography), high-resolution ultra-
sound biomicroscopy, fluorescein angiography, com-
puterized tomography, magnetic resonance imaging,
and fine-needle aspiration biopsy.
A. HISTORY
In our practice, a past medical history and the
ophthalmic examination combined with ultrasonog-
raphy provides enough information to determine the
diagnosis and management in the majority of cases
of anterior segment tumors. Age, sex, and race are
significant aspects of the medical history. A history of
non-ocular cancers must be investigated: primary
tumor organ location, type, treatment, and history of
metastasis. A past ocular history of ocular trauma,
surgery, and inflammation is important. Patients’
symptoms at the time of diagnosis (e.g., blurred
vision, floaters and flashes, pain) are recorded.
B. CLINICAL EXAMINATION
Slit lamp biomicroscopy and gonioscopy are essen-
tial to clinical evaluation of anterior segment tumors.
Inspection of the eyelids, conjunctiva, the anterior
chamber angle, and the iris are performed. After
pupillary dilation, an examination of the ciliary body
and lens is completed. Direct, indirect, and contact
lens ophthalmoscopy allow a detailed investigation
ANTERIOR SEGMENT TUMORS
of the optic disk, macula, and of the periphery of the
retina. Both ophthalmoscopy and dilated slit-lamp
examination can be used to determine the posterior
extension of an anterior segment tumor.
When a suspected lesion is found, its morphology
is described (plateau, dome, mushroom), surface
(smooth, rough), color (white, yellow, orange, brown,
black) size and width (in millimeters), anatomic loca-
tion (e.g., iris, ciliary body), quadrant(s) or clock
hours affected (superior, nasal, inferior, temporal),
anteroposterior location (e.g., collarette, pupillary
margin, mid-iris, and/or iris root) as well as involve-
ment of surrounding structures (e.g., episcleral senti-
nel vessels, invasion of the angle, and/or iris root).
Secondary uveitis, sector cataracts, dislocated lens,
scleral invasion and posterior extension to the cho-
roid are also noted.
Transillumination of the globe should be per-
formed for tumors affecting the ciliary body and ante-
rior choroid. Less pigmented tumors will transmit
light readily, whereas deeply pigmented and tall
tumors induce a scleral shadow. Slit-lamp and gonios-
copic photography can be extremely useful for docu-
menting the tumor’s clinical characteristics, allowing
for accurate determinations of subsequent changes.
C. ANCILLIARY EXAMINATION
There have been recent advances in the diagnosis
and treatment of ocular tumors. The diagnosis of
anterior segment tumors has been greatly enhanced
by fine-needle aspiration biopsy42,43 and high-
frequency ultrasonography.7,97,145,157,184,189,190,191,210,
212,224,281
The use of computerized tomography72,170
and magnetic resonance imaging has also been de-
scribed.1,159,219 Clearly, these tests can help the sur-
geon plan for appropriate treatments.
1. Fine-needle Aspiration Biopsy
The common indications for fine-needle aspira-
tion biopsy are: when less invasive methods cannot
establish the diagnosis, for a presumed metastatic
tumor with an undetectable primary site, and when
the patient requests a histopathology or cytology
prior to treatment.16,75,79,125 Representative material
has been obtained in about 88% of cases (when a 22-
gauge needle is employed).42,279 The most commonly
reported complication is intraocular hemorrhage
with secondary glaucoma.42,120,125 The risk of extra-
ocular seeding can be minimized by transcorneal
sampling.120 When fine-needle aspiration biopsy is
inconclusive, a surgical iridectomy may be per-
formed. Table 1 presents the main types of neoplasia
diagnosed using fine-needle aspiration biopsy.
571
TABLE 1
Reported Uses for Fine-Needle
Aspiration Biopsy16,42,43,75,120,130,125,191,223,258
Melanoma
Metastasis
Melanocytoma
Adenoma
Lymphoma
Leukemia
Retinoblastoma
Epithelial ingrowth
Cysts
2. Ultrasonography
Diagnosis and treatment recommendations are in-
fluenced by tumor location, size, and local ex-
tension.85,86,157,246 Currently used widely, most
ophthalmic ultrasounds employ an 8–10 MHz trans-
ducer. These “low-frequency” transducers were built
to penetrate up to 40 mm, offer a relatively low (200–
300 µm) resolution, and have a critical focus at 25
mm. To optimize imaging of the anterior segment
tumors, 10 MHz transducers are typically combined
with a 1–2 cm “stand-off” or water-bath technique
(Fig. 2).
Pavlin and Foster realized that higher frequency
systems (20–50 MHz) offer better resolution of the
anterior segment tumors.210,212 What has been
termed the “ultrasound biomicroscope” (UBM)
employs a 50 MHz transducer which provides high-
resolution (50 µm) images up to a typical distance
of 3 mm.210–212 This technique provided us with the
first high-resolution ultrasound images of iris
tumors surface, deep margins, and internal reflectivity
(Fig. 3).21,97,189,190,191,210,212 High-frequency ultrasound
has allowed for quantitative follow-up tumor dimen-
sions, more precise determinations of tumor borders,
and a better evaluation of the involvement of the
surrounding structures.72,84,157,190,191,212,224 Advan-
tages of the 20 MHz system are a wider (6–7 mm)
and deeper (4–5 mm) field. We have found that high-
frequency ultrasonography is an indispensable tool
for the diagnosis, measurement, and follow-up of an-
terior segment tumors.
Three-dimensional high-frequency ultrasonogra-
phy (3DUS) is just beginning to become available for
anterior segment tumors (Fig. 4).145 There have been
several investigative studies, but no commercial
system is available at the time of this review. In sum,
3DUS allows retrospective analysis of the entire
tumor volume.227 It will enable volumetric and topo-
graphic studies of the tumor and help define its rela-
tionship with surrounding structures (including
radioactive plaques).56,94,102
572 Surv Ophthalmol 48 (6) November–December 2003
Fig. 2. Left: Slit-lamp photography shows a ciliary body melanoma indenting the lens and inducing a cataract (arrow).
Right: Water-bath low-frequency ultrasound (10 Mhz) image of the cystic ciliary body melanoma (arrow). C ⫽ cornea;
CB ⫽ contralateral ciliary body; ON ⫽ optic nerve.
3. Fluorescein Angiography
Fluorescein angiography is performed for atypical
anterior segment tumors to help establish the diagno-
sis.14,27,62 The vascular pattern of the tumor is judged
in relationship to the normal vascular pattern of
the iris. In practice, the tumor’s vascular network can
be hyperfluorescent (inside and around the edges of
the tumor). If fluorescein leakage is noted at a site
and remote from the tumor, occult or multifocal
Fig. 3. A ciliary body melanoma with anterior chamber
extension. A 50-Mhz high-frequency ultrasound shows how
the tumor (Tu) has eroded through the iris root (small
arrows). The anterior chamber is blunted (solid arrow-
head). There is an extension of tumor behind the lens
(open arrow). (Reprinted from Marigo et al190 with per-
mission of Archives of Ophthalmology.)
MARIGO AND FINGER
tumors should be suspected. In general, a disorga-
nized vasculature exhibiting gross leakage dye is sug-
gestive of malignancy.62,220
4. Computerized Tomography and Magnetic
Resonance Imaging
Computerized tomography and magnetic reso-
nance imaging can be helpful in the study of scleral
involvement and extrascleral extension of the ante-
rior segment tumors.1,134 They can be used to differ-
entiate between solid tumors and certain benign
conditions, such as foreign bodies, massive hemor-
rhage, and cystic lesions.135,152,170,202 However, vari-
ous solid intraocular tumors cannot be differentiated
Fig. 4. Three-dimensional ultrasound of an anterior seg-
ment tumor (courtesy of Ray Iezzi, MD). S ⫽ sclera;
Tu ⫽ tumor; C⫽ cornea.
ANTERIOR SEGMENT TUMORS 573

from one to another based on computerized tomog-
raphy and magnetic resonance imaging features.219
D. GUIDELINES TO DIFFERENTIATE BENIGN
FROM MALIGNANT LESIONS
Table 2 presents some basic clues, which must be
understood only as general guidelines, that help dif-
ferentiate between benign and malignant anterior
segment lesions.
Benign lesions tend to be flat or slightly elevated;
they do not distort the iris stroma and they should not
grow.19,40,63,118,241,274,286 Multiple or bilateral lesions
are more commonly benign.
Malignant melanomas, however, generally pres-
ents as a solitary nodular lesion located in the lower
half of the iris.286 They tend to be unilateral and
they can be associated with abnormal vascularization,
ectropion uvea, pupillary distortion, sector cataract,
pigment dispersion, and glaucoma (Fig. 5).39,45,63,118,
150,157,190
are usually small, relatively avascular or bilateral le-
sions. Like choroidal nevi, small ciliary body tumors
can be proven to be benign over time. The absence
or presence of tumor growth can be established by
serial high-frequency ultrasound examinations.
However, ciliary body tumors are more likely to be
malignant if they induce a sector cataract, lenticular
astigmatism, extrascleral extension, or a visual field
defect.282 They can be darkly pigmented or amela-
notic with evidence of vascularization. Documented
growth and pigment dispersion are strong predictors
of malignancy.136,251 These tumors can also cause an-
terior displacement and/or infiltration of the iris
(Fig. 3). Table 2 summarizes signs suggestive of malig-
nancy including the following: dilated episcleral “sen-
tinel” vessels, anterior bulging of the iris, iris
infiltration, pupillary distortion, ectropion uveae,
sector cataracts, displacement of the lens, and pig-
ment dispersion.153,244

Anterior segment tumors can distort normal tis-

sues. This is evidenced by tumor-induced eversion of III. Treatment
the iris pigment epithelium onto the iris surface (e.g., This section reviews the various methods of treat-
ectropion uveae), compression of adjacent tissues, ment available to those physicians who care for pa-
and iris displacement. Pigment dispersion and tumor tients with anterior segment tumors. In practice,
infiltration can be visualized on the corneal endothe-
these treatments are employed based on the type
lium, the lens surface, the iris stroma, the trabecular
of tumor and condition of the eye. Specific treatment
meshwork, and ciliary processes.136 Secondary
options for each tumor subtype are discussed in the
glaucomas are typically either melanomalytic (due to
sections written for those tumors.
pigment dispersion) or due to tumor growing within
In general, treatment of anterior segment tumors
(occluding) the aqueous out flow system.45,68,247,251
has trended toward ocular conservation. The trends of
If the tumor causes a sector cataract, it is typically
treatment include observation, local resection, and
in the same quadrant as the tumor. Malignant iris
neoplasms also cause heterochromia, spontaneous radiation therapy. Enucleation is typically reserved for
hyphema, and chronic uveitis.39,47,81,158,286 large tumors or eyes with untreatable glaucoma. As
Because ciliary body tumors are hidden by the new modalities of therapy offered lower morbidity
iris, the clinical differentiation between benign and when compared with enucleation, more physicians
malignant tumors is more difficult. Benign tumors and patients have opted for eye and vision-sparing
treatments.59,84,96,129,214,248
TABLE 2
General Clinical Differentiating Characteristics: Between
A. OBSERVATION FOR CHANGE
Benign and Malignant Anterior Segment Tumors
Lesions that represent little immediate threat for
More Likely
Feature More Likely Benign Malignant survival or morbidity can be observed for growth.10,136
Most iris tumors and small melanocytic lesions of the
Elevation Flat or slight elevated Nodular
ciliary body fall in this category. Lesions selected for
Number One or more lesions Solitary
Laterality Unilateral or Bilateral Unilateral observation must be well documented to allow objec-
Size ⬍3 mm ⬎3 mm tive follow-up evaluations. High-quality photographs
Growth No Yes are used to document the surface characteristics of
Vascularization No Yes the tumor (Fig. 5). Tumor size, boundaries, and
Ectropion uveae No Yes
Iris infiltration No Yes thickness are typically determined by transillumina-
Pupillary distortion No Yes tion and ultrasound examination.84,184,210,224 Treat-
Cataract No Yes ment should be considered if an anterior segment
Sentinel Vessels No Yes tumor demonstrates growth or is the cause of second-
Glaucoma No Yes
ary glaucoma.
574 Surv Ophthalmol 48 (6) November–December 2003
Fig. 5. Risk factors for tumor growth include abnormal vascularization (top left), ectropion uvea (top right), pupillary
distortion (bottom left), pigment dispersion (bottom right) and sector cataract.
B. LOCAL RESECTION
1. Iridectomy
Sector iridectomy involves removing the tumor and
a margin of normal appearing iris. The main compli-
cations are glare, hyphema, cataract, infection, and
IOP fluctuations.18,52,199
When the tumor is located near the iris root, ciliary
body extension must be considered.69,131 Gonios-
copy, transillumination, and ultrasound examination
may be helpful before and after pupillary dilation. If
the ciliary body is affected, iridocyclectomy can be
performed.18,69,131,188,290 Heterochromia iridis or
multiple iris tumors suggest a more diffuse process
or ring melanoma.
2. Lamellar Sclerouvectomy
In lamellar sclerouvectomy, the tumor base is de-
lineated and a free margin of 2–3 mm is out-
lined.59,69,110,131 A scleral flap is prepared over the
outlined area. The deep scleral lamella is incised
down to the choroid and the tumor is lifted with the
sclera and dissected from the retina.
The main indications for lamellar sclerouvectomy
are the ciliary body and iridociliary tumors that are
not supposed to respond well to brachytherapy such
as the adenoma and the adenocarcinoma of the non-
pigmented ciliary body epithelium.96,175,253 The
MARIGO AND FINGER
main contraindications are: involvement of more
than one-third of the ciliary body or anterior cham-
ber angle, significant extraocular extension, and
contraindication for hypotensive anesthesia such
as previous cerebral thrombosis or carotid artery
occlusion.59,110
The main complications observed involve vitreous
and choroidal hemorrhage, retinal detachment,
cataracts, and residual tumor.59,110,226 To reduce the
incidence of hemorrhage, extensive preoperative
photocoagulation is applied as to encircle the tumor.
Then hypotensive anesthesia is employed, followed by
diathermy of the choroid around the tumor and
closure of associated vortex veins when exposed.
2. Full-thickness Eye-wall Resection
This surgery is performed in a similar way to lamel-
lar sclerouvectomy but tumor is resected en bloc.
The excised tissue includes all the layers of the eye
wall beneath the tumor and includes the overlying
retina.214–216, 235 A tectonic scleral or corneoscleral
graft is used to repair the defect. A vitrectomy is
carried out at the end of the surgery to remove any
vitreous incarceration or blood from the excision
site. The complications are similar to those observed
for lamellar sclerouvectomy.215,226
ANTERIOR SEGMENT TUMORS
C. RADIATION THERAPY
There are two modalities of radiation therapy avail-
able for the treatment of anterior segment melano-
mas.84–86 Brachytherapy consists of the application
of radiation over short distances (radioactive
plaques). Teletherapy consists of the application of
radiation from a distance (typically proton-beam
irradiation).57,83,86,108,178,284
1. Brachytherapy
Currently used brachytherapy sources can be di-
vided in two classes of radionuclides (gamma or beta
emitters).83,86 The most commonly used radiation
devices for treatment of intraocular melanomas are
composed of radioactive seeds affixed within a gold
shell (plaque). Iodine-125 (125I) and palladium-103
(103Pd) seeds are currently used widely for brachy-
therapy applications around the world.83,95 In 1986,
125
I plaques were selected by the Collaborative Ocular
Melanoma Study (COMS) and have been widely used
for over 20 years.26,140,198,208,221,240
Palladium-103 has been used to treat iris and ciliary
body melanomas.83,91,92,95,98 Research has demon-
strated that 103Pd offers the same local control rates
as 125I along with a more favorable intraocular and
orbital dose distribution for most tumors.90–92,95,98
Among the beta emitters, Ruthenium 106 (106Ru) is
the most frequently used eye-plaque.13,58,162,163,181–
183,266–268,284
Brachytherapy consists of suturing the radioactive
plaque as to cover over the tumor’s base and a tumor-
free perimeter of 2–3 mm.84,86 The device is typically
left in place for 4–7 days while the tumor receives
the prescribed dose of radiation. When the device
is removed, there is no radioactive residue.
Follow-up visits are conducted at 3–6 months inter-
vals.86 At each visit the tumor is evaluated for growth
or shrinkage. While its clinical appearance is com-
pared to photographic documentation from prior
visits, tumor dimensions (boundaries and thickness)
are usually compared with previous ultrasound mea-
surements.84,224 If tumor growth is observed, treat-
ment failure is considered and additional treatments
can be performed.
The main complications observed in plaque radia-
tion therapy are keratopathy, cataracts, scleral necro-
sis, rubeosis iridis, neovascular glaucoma, radiation
vasculopathy, and radiation neuropathy.84,86,129,248
Our clinical experience suggests that posterior radia-
tion complications are relatively uncommon after
103
Pd plaque radiation therapy of anterior uveal
melanomas.84,85
When comparing local resection and plaque radia-
tion therapy, the use of plaques all but eliminated
complications typically associated with intraocular
575
surgery (hemorrhage, retinal detachment, endoph-
thalmitis) while increasing the size of the treated
zone (margins) to include 2–3 mm of surrounding
normal appearing tissue. On the other hand, as com-
pared to local resection, radiation is associated with
a higher incidence of cataract and neovascular glau-
coma. Although no prospective randomized or case-
controlled studies have compared local resection to
plaque radiation therapy, two phase I clinical trials
suggest that plaque radiation therapy is as effective
or better for local control and visual function.84,85,248
We prefer plaque brachytherapy as treatment for
most iridociliary melanomas. Though adjunctive
thermotherapy has been proposed to increase the
tumor sensitivity to radiation, few patients with
anterior segment tumors have been treated with adju-
vant hyperthermia.50,87–89,91,92,99–101,176,225,229
2. Charged-particle Irradiation
Charged particles such as protons or helium ions
move through matter in almost straight lines, slowing
and eventually stopping at a distance.41,66,86,121,123,177
The amount of radiation deposition increases as the
charged particle slows and interacts with electrons in
the tissue.86,121 Thus, there is a dose increase or “star”
in the end of the particle’s path.
Charged-particle irradiation is effective to control
most anterior segment melanomas with a reasonable
chance for retention of vision and eye.73,123 Com-
pared to brachytherapy, charged-particle radiation
therapy delivers more irradiation to most anterior
segment and adnexal structures.57,86,87,108,284 The
main complications are eyelash loss, punctal occlu-
sion, keratopathy, cataracts, iris neovascularization,
neovascular glaucoma, and rarely, radiation vasculo-
pathy and neuropathy.36,86,108,122,284 The relatively
high incidence of radiation-associated neovascular
glaucoma, cataract, and keratitis sicca are serious con-
cerns. The clinical care of the irradiated eyes is per-
formed in a similar manner to those treated with
plaques.
D. ENUCLEATION
Enucleation remains the standard treatment for
tumors too large to be managed by local resection
or irradiation, for eyes with intractable glaucoma,
and for tumors unresponsive to radi-
ation.68,73,86,93,96,126,133,200 In treatment of large cho-
roidal melanomas, COMS evaluated the use of 20
Gy of preoperative external beam radiation therapy
(PERT) to sterilize any cells that might be liberated
during enucleation surgery.9,198,200 However, they
found that that PERT did not provide any survival
benefit in these cases. From this data it appears that
576 Surv Ophthalmol 48 (6) November–December 2003 MARIGO AND FINGER

enucleation does not increase the incidence of
metastasis.198
IV. Melanocytic Tumors of the Iris and
Ciliary Body
A. NEVUS
1. Iris Nevus
Iris nevi present as focal areas of iris pigmentation
that are flat or slightly elevated. However, some iris
nevi can grow and infiltrate the iris and other anterior
segment structures. When iris nevi present with iris
infiltration, iris distortion, ectropion uveae, and
sector cataract they must be differentiated from iris
melanomas.136
High-frequency ultrasonography has been em-
ployed to help measure and follow suspicious iris nevi
for evidence of growth.190,210,212,224 More typically,
iris nevi appear as a low reflective surface plaque
overlying a thickened iris stroma. Nevi close to the iris
root can present a bowed appearance.190,210,212
Other ultrasonically defined morphologic fea-
tures include: a fusiform thickening of the iris; a
diffuse elongated thickening of the iris; a focal thick-
ening of the iris surface with a distinct, sharp border
between the lesion and the iris, the so called “stuck-
on appearance,” and a collar-button shape similar to
that of choroidal melanomas (Fig. 6).157,190,210,212
In these cases we use high-frequency ultrasound
to define the boundaries of a lesion as well as its
shape and internal reflectivity. It is our practice to scan

Fig. 6. Risk factors for tumor growth as revealed by high-frequency ultrasonography. Top: Invasion of the ciliary body by
low reflective tumor as seen on longitudinal (left) and transverse sections (right). Bottom: Transverse section of an iris
melanoma reveals disruption of the highly reflective iris pigment epithelium (left), and pigment deposition (right) on
the corneal endothelium (best seen on the inferior iris).
ANTERIOR SEGMENT TUMORS 577

iris tumors in radial and cross sections. This allows

for both a transverse and longitudinal assessment
of tumor thickness and extent. Transverse imaging of
the ciliary body can reveal occult infiltration (Fig. 6).
Iris freckles are caused by increased pigmentation
of the anterior border layer melanocytes (without
an increase in the number of cells). Therefore, they
present as flat plaques of increased pigmentation of
the iris surface without alteration of the iris architec-
ture.153 In contrast to the iris nevus, iris freckles have
no associated thickening or nodule formation.
In neurofibromatosis type I, the iris may contain
several lightly pigmented, nodular nevi in a diffuse
distribution (Lisch nodules).32,153,241 These lesions
are characteristically bilateral. Other ophthalmic
manifestations of neurofibromatosis type I include
eyelid neurofibromas, which if in the upper lid may
cause a typical “S-shaped ptosis,” prominent corneal
nerves, congenital ectropion uveae, glaucoma, and
choroidal hamartomas. Orbital features include optic
nerve glioma, neural tumors, and spheno-orbital
encephalocele.
The Cogan–Reese syndrome is characterized by
small, darkly pigmented, pedunculated nodules that
can take either a diffuse or focal distribution on the iris
surface (Fig. 7).49,70,207,239 Cogan–Reese syndrome
is unilateral. Other anterior segment findings in-
clude essential iris atrophy, endothelial corneal ab-
normalities which may lead to corneal edema
(Chandler’s syndrome), peripheral anterior synech- Fig. 7. Top: Slit-lamp photography demonstrates the multi-
ple iris nevi consistent with Cogan-Reese syndrome. Bottom:
iae, and secretion of a new Descemet’s membrane. a unique high-frequency ultrasound demonstrates the nevi
Proliferation of an endothelial membrane over the iris contiguous with the iris stroma and an intact iris pig-
and the anterior segment angle structures will induce ment epithelium.
glaucoma.49,70,207,239
The Nevus of Ota affects women in 80% of cases or iridocyclectomy) can be performed. Iris freckles
and generally becomes evident during adolescence and nevi are benign lesions and are not considered
or early adulthood.104,132,209,231,262,270 It is typically to be a risk factor for melanoma metastasis. However,
unilateral and associated with pigmentation of skin patients with the Nevus of Ota are at risk for uveal
within the distribution of the first and second and central nervous system melanoma and should
branches of trigeminal nerve (including the skin be monitored.
of the lids and periorbital skin). The Nevus of Ota
is also associated with increased pigmentation of 2. Ciliary Body Nevus
the episclera, sclera, uvea and rarely, the conjunc- Ciliary body nevi are relatively infrequent.203 Due to
tiva. The iris presents a sector or diffuse hyperpig- their occult location (behind the iris), they are usually
mentation resulting in heterochromia. The angle is missed during routine examination. Typical ciliary
heavily pigmented with persistence of pectinate body nevi present as small, discoid, relatively avascu-
ligaments. Secondary glaucoma and uveal melanoma lar, and pigmented lesions.
can be associated with the Nevus of Ota.104 Orbital Ciliary body nevi are characterized by thickness.
and intracranial melanomas have also been Serial high-frequency ultrasound evaluations (over
reported.132,165 time) should reveal no change in basal diameter
Iris freckles and nevi do not need to be treated. or thickness. If growth is documented, the diag-
Suspicious lesions are typically monitored for growth nosis of ciliary body melanoma should be consid-
(photography and high frequency ultrasonography) ered. If the patient has oculodermal melanocytosis
prior to considering treatment. If growth is defini- (Nevus of Ota) and a ciliary body nevus, that tu-
tively documented, trans-corneal fine-needle aspira- mor must be watched closely for malignant
tion biopsy or excisional biopsy (sector iridectomy transformation.209,261,262
578 Surv Ophthalmol 48 (6) November–December 2003
B. MELANOMA
1. Iris Melanoma
Malignant melanomas of the iris comprise between
5–10% of all uveal melanomas. They are considered
the most common primary iris malignancy.153 The
age of presentation is typically in the fifth and sixth
decades of life and there is no sex predilection. The
majority of iris melanomas develop from a pre-
existing iris nevus and within the iris stroma.153
Iris melanomas most frequently originate in the
peripupillary iris, followed by the midzone and less
frequently in the iris periphery.118,274 The inferior
quadrant is most frequently affected, followed by the
temporal, nasal, and superior quadrants. It is interest-
ing to consider that the inferior quadrants are typi-
cally least shaded from sunlight. Most of the iris
melanomas are composed of spindle cells.125,148,149
Iris melanomas most commonly present as a soli-
tary nodular tumor.39,136,157,190 Other less frequent
patterns of presentation are the diffuse and “tapioca”
colored forms of iris melanoma.19,31,40,63,220,286 As with
other uveal melanomas, the degree of pigmentation
can vary from heavy diffuse pigmentation, variable
pigmentation, or amelanotic.
Clinical features characteristic of malignant mela-
noma of the iris melanomas include tumor vasculari-
zation, ectropion uvea, pupillary distortion, pigment
dispersion, sector cataract, and glaucoma (Fig.
5).136,153,286 Lesions with largest basal diameter
greater than 3 mm are considered suspicious. The
ability to visualize tumor blood vessels is dependent
on tumor pigmentation. Vessels tend to be easily
visible in amelanotic melanomas and hardly detect-
able in pigmented ones. A distortion of normal tis-
sues around the tumor and displacement of the
iris pigment epithelium onto the iris surface (ectrop-
ion uveae) can be a result of tumor growth, contrac-
tion, or neovascularization. Pigment dispersion into
the anterior segment can be present and visualized
on the corneal endothelium, the iris surface and the
trabecular meshwork. Pigment dispersion or tumor
extension to the anterior chamber angle can induce
secondary glaucoma.45,251 If the tumor induces a
sector cataract, it is typically in the same quadrant as
the tumor. Other findings including heterochromia,
spontaneous hyphema, and chronic uveitis have been
associated with iris melanoma.
Iris melanomas (as imaged by high frequency ultra-
sound) are typically low to medium reflective and
nodular arising from the iris surface or a medium to
highly reflective thickening of the iris stroma (Fig.
6).157,190,212 Displacement of iris surfaces results in a
bowed profile, which indicates infiltration of the iris
stroma.190 This is a common finding in that the stroma
is typically involved. Tumor infiltration of the iris
MARIGO AND FINGER
pigment epithelium can be seen by ultrasonography
and suggests malignancy. Lastly, the presence of
acoustically empty or cystic spaces in the iris stroma
have been found to be blood vessels.190 Small projec-
tions attached to the main tumor may extend to the
angle and ciliary body, and change the prognosis for
metastasis (Fig. 6).84,210
Fluorescein angiography can demonstrate the vas-
cular pattern of the lesion but typically is of little
clinical help.14,27 Conventional water-bath ultra-
sound examination also provides additional infor-
mation but with limited resolution.146
Diagnosis of iris melanoma depends on features
that are suggestive of tumor malignancy including
tumor size, distortion of iris, ectropion uveae, intrin-
sic vascularity, and sector cataract (Fig. 5). However,
documented growth is the most important feature
for diagnosis. Suspicious lesions should be photo-
graphed and measured with ultrasound, then care-
fully followed up at 3–6 months intervals monitoring
for growth. We suggest high-quality slit-lamp and
gonio-photographs together with either a video of the
high-frequency ultrasound and/or representational
cross sectional images with measured tumor height
and thickness. Fig. 6 shows how the ciliary body in
the quadrant of the tumor can be visualized. The
other quadrants should also be examined looking
for evidence of a ring melanoma. Clearly, changes
in tumor size and internal reflectivity suggest
malignancy.190,210,212
The main differential diagnosis of the iris melano-
mas include iris nevi, iris cysts, leiomyoma, metasta-
ses, and juvenile xanthogranuloma.255,274 However,
differentiation between benign and malignant le-
sions, mainly those located in the iris, is still a chal-
lenge. Histopathologically benign lesions such as nevi
can grow and invade adjacent tissues and even recur
after excision.148,149,286 Therefore, the definitive diag-
nosis is often both clinical and histopathologic.
Sector iridectomy is the procedure of choice for
suspected malignant melanoma of the iris, and may
be combined with cataract surgery when neces-
sary.118,136,157,190,286 Lesions which extend to or invade
the ciliary body are treated with iridocyclectomy or
ophthalmic plaque radiation therapy.84,188,248 For dif-
fuse tumors with intractable glaucoma, enucleation
is commonly employed.64,126,200,250
Iris melanomas are usually diagnosed early. This
is probably because the iris is visible and the
tumors are easily noticed. Most are spindle-cell mela-
nomas typically located far from points of egress from
the eye, therefore iris melanomas rarely metasta-
size.136,250,286 The mortality rate for iris melanomas
has been reported to be 4–8%.136
ANTERIOR SEGMENT TUMORS
2. Ciliary Body Melanoma
Uveal melanomas are less commonly centered at
the ciliary body compared to either the iris or cho-
roid.139 It is reported to commonly appear in the
sixth decade of life, and is very rare in African and
Asian patients.217,282 Unlike iris and choroidal mela-
nomas, they may be primarily located in any quadrant
of the ciliary body.190 They can be darkly pigmented
or amelanotic with apparent vascularization.
Ciliary body melanomas can present when they are
quite large. This is due (in part) because they are
hidden from view (behind the iris). When they be-
come large enough to cause symptoms, they have
grown to displace the iris, lens, or into the visual axis
(Figs. 2 and 3). Their access to emissary canals may
predispose these tumors to anterior or posterior ex-
trascleral extension (Fig. 1).244,245,282
Clinical signs suggestive of the malignancy include:
engorged episcleral “sentinel” vessels, anterior bulg-
ing of the iris, iris infiltration, pupillary distortion,
ectropion uveae, sector cataract, displacement of the
lens (irregular astigmatism), pigment dispersion, ret-
inal detachment, and extrascleral extension.3,153,282
Many large tumors extend in to the anterior choroid
and iris, making it difficult to determine their ana-
tomic origin.
Diffuse or retino-invasive melanoma is reported
to be a distinct clinical presentation of ciliary body
melanoma. Often confused with ring melanoma, it
differs in that tumor growth is slow; the neoplastic
cells adhere and proliferate to the vitreous, hyaloid
interface, and local retinal surface infiltrating the
nearby neurosensory retina and optic nerve but not
the choroid and the non-adjacent retina.160
Extrascleral extension has been associated with
a greater incidence of metastatic disease.233,245,246
The tumor typically exits through scleral emissary
channels or by direct infiltration of the sclera (Fig.
1).190,212,246 Other lesions that may simulate cil-
iary body melanoma with extrascleral extension
include staphyloma, occult foreign body, and
melanocytoma.174,282
Ultrasound examination continues to be the stan-
dard method to define the tumor’s posterior
extent.211,227 Low-frequency, B-scan evaluations of cil-
iary body melanomas typically reveal a homogeneous
collar-button or dome-shaped mass with low internal
reflectivity. Internal echolucent areas are common.
High-frequency ultrasound examinations offer
unique images of ciliary body melanomas.84,190,210, 212
High-frequency examination can be used to measure
tumor size, extension into the iris root, and lenticular
displacement (Fig. 6).84,184,224 Although basal dimen-
sions of most ciliary body melanomas can be defined
by transillumination, tumor shadows can merge with
579
the ciliary body band. In cases of iris and ciliary body
melanoma, it is important to perform sequential
clock-hour ultrasonographic imaging of the anterior
segment to determine the lateral tumor margins
within the ciliary body (Fig. 6). This technique is of
particular value in assessment of ring melanomas.190
The internal reflectivity of ciliary body melano-
mas (as imaged by high-frequency ultrasound)
is moderate.210,212 Iris stromal invasion is repre-
sented by a change in the echogenicity of the af-
fected area.190 We have found that disruption of
the iris pigment epithelium suggests malignancy
(Fig. 6).190,224
Invasion of the anterior chamber angle has been
observed as a loss in the normal acute shape of the
angle, which assumed a convex or linear shape (Fig.
3).190 In more advanced cases, a moderately echolu-
cent tissue can be seen in the anterior chamber.
The scleral spur and the Descemet’s membrane are
important landmarks when evaluating the trabecular
meshwork and cornea for infiltration by tumor
cells.190 This information about the intraocular distri-
bution of the tumor is particularly important when
considering radioactive plaque therapy.84 Proper
plaque size and position (relative to the tumor and
other landmarks) can limit unnecessary irradiation
of unaffected structures.84,86,212
Posterior extension of anterior uveal tumors can
be determined by ultrasound.190 These tumors can
extend to the lens equator or lenticular surface.190
The tumor can eventually encroach and dislocate
the lens, disrupting the lenticulopupillary axis.190
In these cases, high frequency ultrasound can help
plan treatment, by demonstrating if complete local
resection can be performed.
Serous retinal detachment are associated with
uveal melanomas.246 The detachment can be imaged
as a highly echogenic line delimiting a small fluid-
containing sonolucent space.190 Relatively small
and previously obscure secondary serous detach-
ments have been imaged with high frequency
ultrasound.190
Cavitation within ciliary body tumors must be dis-
tinguished from large blood vessels within the tumor
and slit-like spaces of supraciliary effusion that can
be found in ciliary body melanomas.7,54,210,212 These
cavities may be empty or they may contain erythro-
cytes, serous fluid, pigment-laden macrophages, or
necrotic debris.180,242
Scleral invasion appears either as a localized loss
of integrity in the chorioscleral interface, a decrease
in scleral reflectivity, or as a sonolucent line that is
thought to represent tumor within an emissary
canal.190,211,212
Differential diagnosis of ciliary body melanoma
includes ciliary body cysts, neuroepithelial and im-
plantation cysts, metastasis, leiomyoma, schwannoma,
580 Surv Ophthalmol 48 (6) November–December 2003
neurofibroma, melanocytoma, adenoma of the cili-
ary body epithelium, adenocarcinoma, pseudo-
epitheliomatous hyperplasia (Fuch’s adenoma)
medulloepithelioma, and lesions that simulate extra-
scleral extension such as staphyloma and occult for-
eign body.
Treatments of ciliary body melanomas continue to
evolve. Primarily due to their relatively large size at
presentation, past ciliary body melanomas were typi-
cally treated by enucleation. In more recent years, the
combination of early detection and new treatments
has allowed preservation of both eyes and vision.
High-frequency ultrasound has also allowed for
detection of small ciliary body melanomas. For
example, we have detected small ciliary body melano-
mas associated with minimal iris root invasion (pres-
enting as a peripheral iris nevi). Another was found
beneath an episcleral sentinel blood vessel. We also
image patients with idiopathic heterochromia
and/or ectropion uveae. Like small choroidal mel-
anomas, most small ciliary body tumors can be ob-
served for evidence of growth prior to treatment.
Early detection of small tumors enables the use of
local resection techniques or decreases the radiation
dose required to sterilize these tumors.
Plaque radiation therapy is particularly effective in
treatment of iris and ciliary body melanomas.8,13,84,
86,129,248
Finger et al reported that 91% of patients
maintained within 2 lines of their pretreatment visual
acuity at a mean 34.4 months (range 9–117
months).84 Though 71% of the phakic patients devel-
oped radiation cataract, only one developed maculo-
pathy (CME).84 In other series, reported
complications have included: radiation retinopathy,
vitreous hemorrhage, corneal lesion, and scleral
necrosis.24,86,248
Charged-particle external beam “proton” radio-
therapy is also used for ciliary body melanomas.73,86,
123,153
Because the targeted zone (Bragg–Peak) must
be moved into the anterior segment for this tech-
nique, the incidence of eyelash loss, dry eye, keratopa-
thy, neovascular glaucoma, and cataract are
increased.86,121
Partial lamellar sclerouvectomy is typically em-
ployed for tumors no greater than 15 mm in diame-
ter.59,69,110,131 It involves the resection of the
neoplasm with a rim of healthy uvea under a scleral
flap. However, it is a relatively hazardous technique
with possible complications, including cataracts, vit-
reous hemorrhage, retinal detachment, and tumor
recurrence.58,60,226 Secondary enucleations are not
uncommon.8,61 Though (with this technique) tumor
seeding of the orbit is inevitable, local orbital re-
currences have not been reported.226
Enucleation is still the treatment of choice for
extra-large melanomas or large melanomas in eyes
MARIGO AND FINGER
without useful vision.86,200 These tumors are not typi-
cally irradiated or resected due to the large tumor
volume. When the tumor fills most of the intraocu-
lar volume, the amount ocular radiation is so large
that the eye does not tolerate standard doses.
Lastly, the COMS has shown that 20 Gy pre-enucle-
ation external beam radiation therapy failed to
decrease the incidence of metastatic choroidal
melanoma.51
Exenteration (removal of the eye and all orbital
tissues) has been employed for cases with massive
extraocular extension.86,200 Smaller extraocular ex-
tensions can be managed by post-enucleation exter-
nal beam radiotherapy. We typically prescribe 50 Gy
(5000 cGy).86 Palliative treatment consists of chemo-
therapy and immunotherapy for patients with
metastasis.5,285
Ciliary body melanomas are thought to be more
malignant with a 5-year survival rate of 59%.66,156,196
Factors that influence the metastatic potential have
been reported to include the following:
1. Cell type: spindle cell melanomas are least likely
to metastasize. Tumors that contain mixed cell-
types, necrotic-areas, and epithelioid-cells carry
a worse prognosis.112–114,139,194,197,243,245
2. Tumor size: larger tumors carry a worse progno-
sis than smaller ones.66,139,196,245 The largest di-
ameter of the tumor is considered to be the
most important predictor of metastasis.
3. Tumor extension: extrascleral tumor extension
increases the risk of metastases.86,245,282
4. Growth patterns: A diffuse growth pattern car-
ries a worse prognosis.31,67,68,247
5. Age of the patient: Patients over the age of 65
years are reported to have a worse prognosis
than the younger patients.112,245,250
3. RING MELANOMA
Ring melanoma of the anterior segment is rare and
can be considered a variety of diffuse melanoma.68,103
It usually presents as a unilateral glaucoma.45 Goni-
oscopy demonstrates infiltration of the anterior
chamber angle structures. The diagnosis can be diffi-
cult unless comparative gonioscopy is performed.
Other findings suggestive of ring-melanoma include
sentinel vessels, ectropion uvea, iris heterochromia,
and cataract.
These cases can be complicated by trabeculec-
tomy.64,126 Trabeculectomy may allow tumor seeding
and should be avoided.126 Clearly, patients with uni-
lateral glaucoma and heterochromia should have
anterior segment high frequency ultrasound exami-
nations. Transcorneal fine-needle aspiration biopsy
ANTERIOR SEGMENT TUMORS
or iridectomy biopsy should be considered. By defini-
tion, ring melanomas have large basal dimensions
and carry a poor ocular and systemic prognosis.
C. MELANOCYTOMA
Melanocytoma is a benign tumor composed of uni-
form, densely pigmented plump polyhedral
cells.43,158 Melanocytomas are rare, more common
in darkly pigmented patients, found after the third
decade of life, and exhibit a slight preference for
females.158,255
Although they commonly present as a peripapillary
tumor, melanocytomas can present as a tumor of
the iris or ciliary body.22,47,54 Most melanocytomas
are stationary and they do not cause symptoms or
secondary complications.158 However, these tumors
can grow, cavitate, shed pigment (cells, melanin).
Melanocytomas can simulate uveal melanoma caus-
ing hyphema, correctopia, or uncontrollable glau-
coma.22,82,158 Extrascleral extension is possible.232,235
There have been several reports indicating that mela-
noma can arise from a melanocytoma.47,141
A suspected melanocytoma may be observed for
growth. If growth is documented the diagnosis of
melanoma must be considered. Fine-needle aspira-
tion biopsy can be helpful for iris melanocytomas.43,75
Local resection is typically performed when docu-
mented growth and tumor shedding risks or induces
secondary glaucoma.22,54,235 There are reports dem-
onstrating that melanocytomas can simulate melano-
mas, and in some of these cases the correct diagnosis
could only established after enucleation.47,141 The
differential diagnosis includes nevi, ocular melano-
cytosis (Nevus of Ota) and adenoma/adenocarci-
noma of the pigmented ciliary body epithelium.
D. ADENOMA AND ADENOCARCINOMA OF THE
PIGMENTED CILIARY BODY EPITHELIUM
Adenoma and adenocarcinoma of the ciliary body
are very rare tumors that are usually differentiated
from melanoma by histopathology.96,124,175,252,253
Adenoma should be suspected when viewing an amel-
anotic ciliary body tumor.96 They tend not to make a
transillumination shadow, and can induce uveitis.96
Ultrasonography demonstrates moderate to high in-
ternal reflectivity with little to no tumor penetra-
tion into the uvea. Fluorescein angiography presents
leakage to the vitreous. Adenocarcinoma of the pig-
ment epithelium should be suspected in women with
persistent uveitis and a melanotic intraocular tumor
that respond poorly to radiation plaque therapy.96
These tumors are typically managed by observation,
local excision or enucleation.
581
V. Anterior Segment Cysts
Cystic lesions occurring within the anterior seg-
ment may be classified as primary or second-
ary.97,155,189,191,254 Primary cysts are of neuroepithelial
origin, whereas secondary cysts may result from im-
plantation, metastasis, parasites or miotic ther-
apy.7,142,144,189,191 Neuroepithelial cysts involve the
pigment epithelium of the iris and the ciliary body.
When large, they may produce focal angle-clo-
sure.269,275 Secondary cysts are more problematic and
may lead to corneal edema, uveitis, glaucoma, and
decreased visual acuity.25,74,97,111,166,234,269 Cysts can
be found in a variety of locations within the anterior
segment and can have different etiologies.7,97,119,210
A. NEUROEPITHELIAL CYSTS
Neuroepithelial cysts are the most common and
are usually located at the iridociliary junction (Fig.
8).7,189,210 They are round or oval with thin walls and
sonolucent contents (as imaged by UBM). It is gener-
ally accepted that the high reflectivity of the cyst
wall is caused by its epithelial cell lining and that its
sonolucent core is compatible with a fluid content
(Table 3).7,189 Cysts restricted either to the iris stroma
or ciliary body are less common (Fig. 8).7,189,210
Iridociliary cysts typically displace the iris root ante-
riorly. This can induce a focal plateau-iris configura-
tion with or without angle-closure.7,11,145,212,269,275
Although single cysts are more common, multiple
cysts are found in at least one-third of cases.189 When
multiple cysts involve more than 180 degrees of the
iris, as it does in 10% of patients, angle-closure glau-
coma may develop.189
B. UVEITIC CYSTS
Uveitis-related cysts are usually bilateral and lo-
cated within the posterior iris and ciliary body epithe-
lium (at the iridociliary junction or anterior ciliary
body).119,189 Most of these iris cysts are associated
with nongranulomatous uveitis and some patients
have been found to be HLA-B27 positive.119,189 Pa-
tients with uveitic cysts tend to be young, with associ-
ated iridocyclitis, and asymmetric (lower) intraocular
pressure. Uveitic cysts can look similar to neuroepi-
thelial cysts when imaged by UBM (Table 3).119,189
C. IMPLANTATION “SECONDARY” CYSTS
Implantation of epithelial cells from the cornea, con-
junctiva, or skin may occur during surgery, perforating
ocular trauma or through a poorly closed surgical
wound.25,48,74,78,97,142,144,164,191,192,213,234,283 Prolonged
postoperative hypotony or incarceration of iris or
lens capsule are risk factors. Although normal aque-
ous is supposed to inhibit growth of these cells, the iris
582 Surv Ophthalmol 48 (6) November–December 2003 MARIGO AND FINGER

Fig. 8. Top left: iris pigment epithelial cysts most commonly present as a raised “tented” iris surface. High-frequency
ultrasonography of these cysts reveals a sonolucent core, displacement of the iris stroma, and focal angle closure (top
right). Middle: sonolucent iris stromal cysts destroy the normal iris architecture (arrow). Bottom: Pearl cysts are composed
of a dense epithelium and filled with mucus and cholesterol crystals.

provides a more than adequate environment for
cell proliferation.179,228
Three types of proliferation are recognized: (a)
pearl cyst, (b) serous cyst, and (c) epithelial down-
growth.97,111,192,193 Pearl cysts are typically small, white
tumors with opaque walls located in the iris stroma
(Fig. 8).97 Pearl cysts were originally described by
Maumanee as having three layers.192 His observations
were confirmed by high-frequency ultrasonography
where round to ovoid tumors were found to contain
three concentric layers.97,191 The external layer has
moderate reflectivity and correlates with the cystic
epithelial lining. The intermediate layer has lower
reflectivity corresponding to degenerated epithelial
cells, mucus and inflammatory debris. Finally, a cen-
tral, highly reflective core has been correlated to
keratinous debris in the center of the cyst contents
and cholesterol crystals derived from degenerative
keratinized cells. Pearl cysts should be completely
excised to prevent mucogenic glaucoma.4,97,168,173
ANTERIOR SEGMENT TUMORS 583

TABLE 3
High-Frequency Ultrasound Findings11,16,21,97,119,169,189,191,211
Cyst Type Cystic Wall Cavity Number Laterality
Neuroepithelial High reflectivity Anechoic Multiple Bilateral
Uveitic High reflectivity Anechoic Multiple Unilateral
Implantation Pearl Medium reflectivity Completely filled with 3 layers: Solitary Unilateral
A medium reflective external
layer, a lesser reflective interm-
ediate layer and a highly
reflective core
Mixed Medium reflectivity Predominantly anechoic partially Solitary Unilateral
filled with medium reflective
material with a highly reflective
core adhered to the cystic wall
Serous Medium reflectivity Predominantly anechoic with sparse Solitary Unilateral
highly reflective dot echoes
Cavitary melanoma Medium reflectivity Anechoic, may have Solitary Unilateral
sparse echoes or multiple
Anechoic ⫽ Acoustically empty.

Serous, translucent cysts are more common and VI. Metastatic Tumors
can erode through the iris and invade the posterior Uveal metastases are the most common intraocular
chamber.191,193 Their growth rate is variable. cancers (Fig. 9).76,81,153 The most frequent intraocu-
Serous cysts of the iris have been found to grow over lar locations for metastatic tumors are the choroid
time, and suddenly become stationary.25,74,191,193 (88%) followed by the iris (9%) and the ciliary body
They tend to develop large tumor diameters that can (2%).249 Metastasis to the eye most commonly origi-
cause iris atrophy by compression.25,191 These cysts nates from the breast in woman and the lung in
are typically imaged as round or elliptic lesions with men.76,81,249,258 Typically, there is a history of mastec-
thick walls and a sonolucent cavity. They can be sep- tomy or lung cancer at presentation.137,249 However,
tate. Dense fluctuating particles have been observed 18–30% of patients have been reported to have no
within cystic cavity.73,189,191 known or discoverable primary tumor.249 Most of
Epithelial downgrowth is usually detected as a thin these tumors are found to be from lung or breast
translucent membrane on the posterior surface of the primaries. Other less frequent sites of origin include
cornea.192,193 A fine gray line can be visualized at the gastrointestinal tract, the kidney, thyroid, and
the borders of the advancing membrane. As the endo- the testes.76,81,249 The prostate is an uncommon pri-
thelium is covered, the cornea becomes edematous mary site.
and neovascularization can present within the cor- Intraocular metastases are typically discovered in
neal stroma. When the membrane extends onto the the choroid, followed by the ciliary body and rarely
iris surface, it appears as a thin translucent film that
changes the normal iris topography.193 A mild irido-
cyclitis can be present.193 Intractable glaucoma may
develop if the membrane grows to cover the trabecu-
lar meshwork.193 High-frequency ultrasound usually
reveals epithelial downgrowth as a moderately echo-
genic membrane on the surface of the iris and adher-
ent to the corneal endothelium.
Implantation cysts are treated when they enlarge
causing a decrease in the visual acuity, glaucoma,
uveitis or cataracts.25,74 Treatment is controversial.
When one or more of these complications is noted,
total excision (as possible) is the best treat-
ment.25,48,74,78,191 Treatment may also require a tec-
tonic graft.25 Other treatments include aspiration of Fig. 9. Metastatic carcinoma from the lung to the ante-
the cyst, and/or laser therapy.34,78,191 rior segment.
584 Surv Ophthalmol 48 (6) November–December 2003
in the iris.17,137,249,258 Anterior uveal metastasis can be
multifocal and/or bilateral. Concurrent pulmonary
and brain metastasis are frequent. Therefore we sug-
gest radiographic imaging studies of the lung and
brain at the time of ocular diagnosis.
Iris metastases typically present as pink or yellow
solitary or multifocal tumors. Like ciliary body mela-
nomas, ciliary body metastasis is generally large at
the time of the diagnosis. Metastasis to either iris or
ciliary body can also present as hyphema, anterior
uveitis or as a pseudohypopyon.81,237
Ultrasonography of metastatic tumors typically
demonstrates relatively high internal reflectivity and
irregular shapes. If the clinical history and medical
workup are negative, we have found trans-corneal fine
needle or incisional biopsy to be most helpful.
Iris and ciliary body metastasis may be treated with
systemic chemotherapy, but often prompt external
beam radiation therapy offers the best hope for pre-
venting secondary glaucoma and a blind and painful
eye.46,137,206 While radiation is helpful, the intraocu-
lar mass should not be irradiated until the primary
is found. In some cases, biopsy of the intraocular
tumor is necessary to diagnose the location of the
primary neoplasm.
Lymphoid and breast metastasis are more likely to
be affected by systemic chemotherapy.285 Several au-
thors suggest that radiation can be postponed to eval-
uate the effects of chemotherapy on an intraocular
tumor. In our experience, this approach is more rea-
sonable in treatment of choroidal metastasis where
secondary glaucoma is not as much of a problem.
Delay is less advisable in treatment of anterior seg-
ment metastasis.
If intractable painful tumor-induced glaucoma
occurs, enucleation or retrobulbar alcohol injection
can occasionally be required. Prognosis for survi-
val after anterior uveal metastasis is often poor and
radiographic imaging of the brain and lungs are
recommended.
VII. Tumors of the Nonpigmented
Ciliary Body Epithelium
A. MEDULLOEPITHELIOMA
Medulloepithelioma is a rare, congenital tumor,
originated mainly from undifferentiated nonpig-
mented epithelium of the ciliary body.138,272 These
tumors are classified as nonteratoid and teratoid
types; the latter contains heterologous tissues. Most
patients present in the first decade of life.138,272
Medulloepithelioma is typically noted to be a
mass lesion in the ciliary body.143,272 Ultrasonographi-
cally, findings of a highly reflective, irregularly struc-
tured tumor with associated cystic changes involving
MARIGO AND FINGER
the ciliary body region are helpful to establish a pre-
sumed diagnosis of medulloepithelioma.109 Medul-
loepithelioma appears hyperintense on T1-weighted
images and hypointense on T2- weighted in MRI
images.219
Although most medulloepitheliomas are benign
tumors, they can contain malignant tissue (similar
in appearance to retinoblastoma) that can be locally
invasive. They can also erode to the anterior chamber
or become externally visible.161 Iris vascularization,
glaucoma, and cataracts may be induced.251,260
When localized, the tumor can be managed by local
resection.161 Recurrence is reported.161 If local treat-
ment is not possible and the tumor erodes the ocular
walls, enucleation is performed.161,200
B. FUCHS’ ADENOMA (PSEUDOEPITHELIOMATOUS
HYPERPLASIA)
Pseudoepitheliomatous hyperplasia is a benign, ac-
quired lesion that arises in the non-pigmented epi-
thelium of the pars plicata of the ciliary body.288 It
is most likely a proliferative disease rather than a
tumor. Histopathologically, the lesion is composed by
a nonvascular proliferation of the non-pigmented
ciliary body epithelium that is rich in acid mucopoly-
saccharides.288 Some surveys have found pseudoepi-
theliomatous hyperplasia in as many as 25% of
older people.288
The tumor has been typically found as an inciden-
tal finding during histopathologic examination of
an enucleated eye. It usually presents as a white glis-
tening lesion arising in the pars plicata of the ciliary
body. Depending on its size, it can cause a focal
narrowing of the anterior chamber angle.288
C. ADENOMA AND ADENOCARCINOMA
OF THE NONPIGMENTED CILIARY BODY
EPITHELIUM
Adenoma and adenocarcinoma of the nonpig-
mented ciliary body epithelium are extremely rare.
Patients are usually diagnosed by the age of 45 years,
and there is no known predisposition for sex and
age.128,172,202,253
They usually present as an amelanotic ciliary body
mass with an irregular or multilobular surface.128,195
These tumors appear to induce or are a result of
intraocular inflammation. Flare and cells in the ante-
rior chamber and anterior vitreous are common.
Other associated findings are secondary cataracts and
lens dislocation (mass effects). Sentinel vessels have
been reported. Like most amelanotic tumors, these
lesions are translucent.128,195 Fluorescein angiogra-
phy presents an early leakage of dye to the anterior
vitreous. Standardized ultrasonography reveals a
high internal reflectivity.128,195 Most adenomas and
ANTERIOR SEGMENT TUMORS
adenocarcinomas of the nonpigmented ciliary body
epithelium are managed by local resection.6,43,172,278
We have found no confirmed cases of metastatic ade-
nocarcinoma of the nonpigmented ciliary epi-
thelium. Therefore, the goals of treatment are to
prevent the complications of secondary uveitis or the
complications of tumor expansion.
VIII. Myogenic Tumors
Leiomyoma of the uvea is an extremely rare iris
or ciliary body tumor.107 Because the smooth muscle
of the iris and the ciliary body is from neural crest
origin, this tumor is considered to be from mesectod-
ermal origin.35,257,290
The leiomyoma occurs in younger patients and
has a predilection for women. Leiomyomas can be
difficult to distinguish from melanomas. However,
there are some features that can help. First, leiomyo-
mas tend to occur in young women. Second, it tends
to affect the ciliary body or the peripheral choroid
and is rare in the posterior choroid. Third, the tumor
seems to grow in the supraciliary or suprachoroidal
space between the uvea and the sclera and it does
not seem to arise from the uveal stroma. Last, leiomy-
oma are relatively amelanotic, and should not pro-
duce a shadow when transilluminated.53
Treatment consists in local resection by lamellar
sclerouvectomy.256 Differently from melanoma, leio-
myoma presents positive immunoreactivity for
muscle markers and negative immunoreactivity for
melanoma-specific antigen and neural markers. Be-
cause they are benign tumors, they too can be closely
followed for evidence of growth prior to resection.
IX. Neurogenic Tumors
A. NEURILEMOMA (SCHWANNOMA)
Neurilemoma (Schwannoma) is a benign tumor
derived from Schwann cells, which wrap around the
axons of peripheral nerves.218 The most frequent
site of occurrence of an intracranial neurilemoma is
the acoustic nerve; however, the tumor may occur
along the 2nd, 5th, 7th, 11th, and 12th cranial
nerves.154 Neurilemoma arising from the ciliary body
or choroid is a very rare tumor.
Clinically, the tumor is confused with amelanotic
melanoma.154,218,263,271 It usually presents as amela-
notic mass that can dislocate the iris root and lens and
infiltrate the angle determining sector cataract and
glaucoma.218 Ancilliary examination such as transillu-
mination, ultrasonography, fluorescein angiography,
CT, and MRI usually cannot rule out melanoma and
therefore the definite diagnosis can only be estab-
lished in histopathologic and immunohistochemical
basis after fine-needle aspiration biopsy.167,218 Treat-
ment consists of local excision of the tumor.167,218
585
B. NEUROFIBROMA
Neurofibroma is a tumor derived from the neural
crest.280 It also arises from the ciliary nerves.32 Neuro-
fibroma of the uvea is very rare and generally related
to neurofibromatosis.32
X. Vascular and Hematogenic Tumors
Anterior uveal hemangiomas have been ob-
served.2,71,115,117,171,236,259 These lesions are usually ha-
martomas and they consist of capillary, cavernous, or
mixed capillary–cavernous hemangiomas.115 Race-
mose angiomas of the iris are rare.259
Hemangiomas usually affect the eyelids and the
orbital region.117,236 Other ophthalmic sites include
the conjunctiva, episclera, retina, and the uveal
tract.15,23,38,117,147,265,273 There are hemangiomatosis
syndromes related to the phakomatosis.117,273,291
Iris hemangioma presents as a vascular lesion or
vascular tuft of the iris surface.15,115,171 Many tumors
initially diagnosed to be iris hemangiomas were later
found to be early melanomas on histopathologic eval-
uation.80 Clearly serial observation of any presumed
iris or ciliary body hemangioma is warranted to rule
out progression to melanoma.80 When the lesion is
documented to grow or when it is associated with
recurrent hyphema, photocoagulation or local exci-
sion can be performed.15,171 Hemangiomas of the
ciliary body are rare.115,147 The lesion can cause
uveitis and eventually grow.147 Ancillary examinations
typically cannot rule out a malignant tumor. Fine-
needle aspiration biopsy may induce hyphema and
vitreous hemorrhage. Depending on its size, the
lesion can be removed by sector cyclectomy.147 Radia-
tion therapy may be a reasonable alternative.187
Leukemia can involve iris and anterior chamber
as the site of primary disease or recurrence.65,185,237
The majority of cases consist of acute lymphoblastic
leukemia (ALL). It may present as an anterior uveitis,
pseudohypopyon, hyphema, elevated intraocular
pressure, and iris nodules or infiltration.12,20,28,
29,33,65,77,201,205,230,238
It is thought that the anterior
chamber may act as a sanctuary for leukemic
cells.28,65,204,237 The malignant cells had probably
been present in the eye since the onset of the disease.
When necessary the diagnosis can be established by
fluid aspiration from the anterior chamber or by fine
needle aspiration biopsy.186,205,230 Treatment typically
consists of systemic chemotherapy and ocular radia-
tion therapy.20,33,65,237
Lymphoma can affect the eye either as a primary
tumor or as secondary metastasis from systemic
disease.276 Clinically, it may present as granulomatous
anterior uveitis, pseudohypopyon, glaucoma, and
586 Surv Ophthalmol 48 (6) November–December 2003
iris nodules and a ciliary body infiltrate.37,55,151,
222,230,277
The tumor may mimic a ring mela-
noma.151 Bilateral involvement has been de-
scribed.287 Iris biopsy is usually performed to establish
the diagnosis.37,276,277 The tumor can be managed
by chemotherapy and adjunctive radiotherapy.37,285
Similar to leukemia, the eye may act as a sanctuary
site for tumor cells during chemotherapy.277
XI. Retinal Tumors
Retinoblastoma involving the anterior segment is
uncommon.93,116,133 But, anterior extension of the
tumor can be seen at the initial examination or
during the follow-up period after therapy (Fig. 10).133
Anterior extension of the retinoblastoma may involve
the anterior chamber, iris, and ciliary body.133 Clini-
cally, it may present as tumor cells fluctuating in
the anterior chamber, pseudohypopion, hyphema,
or white spots on the iris.127,133
Eyes with retinoblastoma in the anterior segment
are typically enucleated. Subsequent chemotherapy
can be employed for occult or proven metastatic dis-
ease.93,285 External beam radiation is used for orbital
or optic nerve extension.93,116,127 Clearly, high-fre-
quency ultrasonography allows for unique views of
anterior retinoblastomas (Fig. 10).
XII. Secondary Glaucomas
Iris pigment epithelial cysts can cause plateau-iris
and focal angle-closure.30,74,269 Despite these find-
ings, we have only seen two cases of angle closure
glaucoma attributable to iris pigment epithelial
cysts.189 Therefore, most of these tumors require no
more than periodic observation. If angle-closure de-
velops, laser or surgical iridotomy can be per-
formed.30 For larger cysts surgical iridectomy may
be required.
Fig. 10. A composite high-frequency ultrasound image of
an anteriorly located intraocular retinoblastoma clearly
demonstrates variable internal reflectivity, highly reflective
calcium deposits (arrows) with no evidence of scleral inva-
sion. S ⫽ sclera; C ⫽ cornea; Pp ⫽ pars plana; Pc ⫽ pars
ciliaris; AC⫽ anterior chamber; Tu ⫽ tumor.
MARIGO AND FINGER
This is not the case with implantation cysts of the
iris. When pearl-cysts rupture they can cause muco-
genic glaucoma, and epithelial proliferations can oc-
clude the natural aqueous outflow systems of the
eye.4,168,173 Implantation cysts should be totally
removed as possible.97
Iris and ciliary body melanomas as well as melano-
cytomas can also induce glaucoma.19,22,40,44,45,47,68,247
In these cases, the tumor can shed cell and pigment
debris, occluding the trabecular meshwork and de-
creasing aqueous outflow. In addition, ciliary body
tumors can infiltrate and thereby occlude the trabec-
ular meshwork or they can induce neovascular
glaucoma.86,251
Iris and ciliary body tumor growth is the primary
indication for treatment. That is because an enlarg-
ing tumor is likely to become increasingly difficult
to treat, more likely to metastasize and induce intrac-
table glaucoma. Iris and ciliary body melanomas have
been treated by observation for growth, local resec-
tion, radiation therapy, and enucleation. Recent evi-
dence suggests that plaque radiation therapy can
be used to destroy iridociliary melanomas without
the risks of intraocular surgery.84
Anterior segment metastasis can also induce a
rapid onset glaucoma. This is typically due to tumor
invasion of the outflow system.251 Though rapid treat-
ment (chemotherapy or external beam radiation
therapy) may be the patient’s best chance to prevent
a blind painful eye. Keep in mind that this tumor
may be the only manifestation of the patient’s pri-
mary disease.
Retinoblastoma can also induce neovascular or
angle closure glaucoma.264,289 Other tumors reported
to cause glaucoma include hemangioma, lymphoma,
leukemia, medulloepithelioma, adenoma of the iris
pigment epithelium, and adenoma or adenocarci-
noma of the ciliary body epithelium.55,117,251,260,273
XIII. Summary
The diagnosis of anterior segment tumors can be
made by with careful ophthalmic examination. High-
frequency ultrasonography and fine-needle aspira-
tion biopsy are recent innovations that have improved
our diagnostic abilities and treatment options. This
review has shown that treatment depends on the
tumor type, location, size, local extension, patterns of
growth, and related complications. The life expec-
tancy and general health of the patient should also be
considered. Our findings are presented to help doc-
tors with their patient evaluations, treatment options,
and informed consent.
Methods of Literature Search
A search of the Medline database was conducted
using the key words tumors, uveal neoplasms, nevus,
ANTERIOR SEGMENT TUMORS
melanoma, melanocytoma, adenoma, adenocarcinoma,
cysts, metastasis, medulloepithelioma, leiomyoma, neurofi-
broma, neurilemoma, hemangioma, lymphoma, retinoblas-
toma, brachytherapy, radiotherapy, eye enucleation.
Additional references were recovered from the bibli-
ographies of the references. References spanned the
period 1990 to 2002. These references were evaluated
for their pertinence to the topic, with special con-
sideration given to articles that detailed the differ-
ential diagnosis of the anterior segment tumors.
Articles that were repetitious or decidedly preclinical
were omitted from consideration.
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Supported by the EyeCare Foundation and Research to Prevent
Blindness, New York, New York, USA. The authors have no proprie-
tary interest in any of the products or equipment mentioned in
this article.
Reprint address: Paul T. Finger, MD, FACS, The New York Eye
Cancer Center, 115 E. 61st St., New York, NY 10021 USA.
E-mail: pfinger@eyecancer.com
ANTERIOR SEGMENT TUMORS 593

Outline 1. Iris nevus

2. Ciliary body nevus
I. Pertinent anatomy and histology of the ante- B. Melanoma
rior uvea
A. Iris 1. Iris melanoma
B. Ciliary body 2. Ciliary body melanoma
3. Ring melanoma
II. Clinical evaluation of anterior segment tumors C. Melanocytoma
A. History D. Adenoma and adenocarcinoma of the pig-
B. Clinical examination mented ciliary body epithelium
C. Ancillary examination
1. Fine-needle aspiration biopsy V. Anterior segment cysts
2. Ultrasonography A. Neuroepithelial cysts
3. Fluorescein angiography B. Uveitic cysts
4. Computerized tomography and mag- C. Implantation “secondary” cysts
netic resonance imaging
VI. Metastatic Tumors
D. Guidelines to differentiate benign from VII. Tumors of the non-pigmented ciliary body ep-
malignant lesions ithelium
A. Medulloepithelioma
III. Treatment B. Fuchs’ adenoma (Pseudoepithelioma-
A. Observation for change tous hyperplasia)
B. Local resection C. Adenoma and adenocarcinoma of the
1. Iridectomy nonpigmented ciliary body epithelium
2. Lamellar sclerouvectomy
3. Full thickness eye-wall resection VIII. Myogenic tumors
IX. Neurogenic tumors
C. Radiation therapy A. Neurilemoma (Schwannoma)
1. Brachytherapy B. Neurofibroma
2. Charged-particle irradiation
X. Vascular and hematogenic tumors
D. Enucleation
XI. Retinal tumors
IV. Melanocytic tumors of the iris and ciliary body XII. Secondary glaucomas
A. Nevus XIII. Summary