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Review Article

Review of cystic and solid tumors of the iris

Carol L. Shields, Patrick W. Shields, Janet Manalac, Chaisiri Jumroendararasame, Jerry A. Shields
Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, PA., USA

Iris tumors are broadly classified into cystic or solid mass inferiorly, D=diffuse configuration, E=ectropion,
lesions. The cystic lesions arise from iris pigment F=feathery margins. The most powerful factors are
epithelium (IPE) or iris stroma. IPE cysts classically diffuse growth pattern and hyphema. Tumor seeding
remain stable without need for intervention. Iris into the anterior chamber angle and onto the iris stroma
stromal cyst, especially those in newborns, usually are also important. The nonmelanocytic iris tumors
requires therapy of aspiration, possibly with alcohol- are relatively uncommon and included categories of
induced sclerosis, or surgical resection. The solid choristomatous, vascular, fibrous, neural, myogenic,
tumors included melanocytic and nonmelanocytic epithelial, xanthomatous, metastatic, lymphoid,
lesions. The melanocytic iris tumors include freckle, leukemic, secondary, and non-neoplastic simulators.
Overall, the most common diagnoses in a clinical series
nevus (including melanocytoma), Lisch nodule, and
include nevus, IPE cyst, and melanoma. In summary,
melanoma. Information from a tertiary referral center
iris tumors comprise a wide spectrum including mostly
revealed that transformation of suspicious iris nevus
iris nevus, IPE cyst, and iris melanoma. Risk factors
to melanoma occurred in 4% by 10 years and 11% by estimating transformation of iris nevus to melanoma
20 years. Risk factors for transformation of iris nevus can be remembered by the ABCDEF guide.
to melanoma can be remembered using the ABCDEF
guide as follows: A=age young (<40 years), B=blood Keywords: Cyst, eye, iris, melanoma, metastasis,
(hyphema) in anterior chamber, C=clock hour of nevus, tumor

Introduction 3680 iris tumors from a tertiary referral center, collected over
a 40-year period.[1]
There is a wide spectrum of tumors of the iris.[1,2] These lesions
range from nevus to melanoma to juvenile xanthogranuloma Iris tumors can be found in all races, but predominates in
to metastasis. There have been only a few published Caucasians. In the analysis of 3680 cases from the United States,[1]
comprehensive series on the full array of iris tumors and most patient race was Caucasian (96%), African American (2%),
series have emanated from pathology laboratories. In 1958, Hispanic (1%), Asian (<1%), and others (<1%). The mean patient
Duke and Dunn recorded 43 cases. In 1963, Ashton found age at presentation was 48 years with range from 2 weeks to
145 cases and, shortly thereafter, Heath published on 232 iris 95 years. The iris tumors occurred in children (≤20 years) in 12%,
tumors.[3-5] In 2012, Shields et al. reviewed a clinical series of young adults (21-40 years) in 21%, mid-adults (41-60 years) in
36% and senior adults (>60 years) in 31%.

Access this article online Iris tumors can be broadly categorized into cystic (21%) or
Quick Response Code: solid (79%) [Table 1]. The differentiation is evident on slit lamp
Website: examination and confirmed on ocular imaging with ultrasound
www.ojoonline.org biomicroscopy (UBM) or anterior segment optical coherence
tomography (AS OCT).
DOI:
10.4103/0974-620X.122269
Imaging features
Imaging of the iris generally involves high-resolution anterior

Copyright:  2013 Shields CL. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which
permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Correspondence:
Carol L. Shields, M.D., Ocular Oncology Service, Suite 1440, Wills Eye Institute, 840 Walnut Street, Philadelphia, PA 19107. Email: carol.shields@shieldsoncology.com

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Shields, et al.: Iris cystic and solid tumors

Table 1: Iris tumor survey for diagnostic category Table 2: Classification of iris cysts
in 3680 cases Primary cyst
Diagnostic Tumor Iris pigment epithelium cyst
category Number of tumors % of all iris Pupillary
N=3680 tumors Mid-zonal
Cystic 768 21 Peripheral
Solid 2912 79 Dislodged
Melanocytic 2510 68 Free floating
Non-melanocytic 402 11 Anterior chamber
Choristomatous 4 <1 Vitreous chamber
57 2 Iris stroma
Vascular
Congenital
Fibrous 2 <1
Acquired
Neural 3 <1
Secondary cyst
Myogenic 2 <1
Epithelial
Epithelial 35 1
Epithelial downgrowth cyst
Xanthomatous 8 <1
Post surgical
Metastatic 67 2
Post trauma
Lymphoid 12 <1
Pearl cyst
Leukemic 2 <1
Drug-induced cyst
econdary 12 <1
Cyst secondary to intraocular tumors
Non-neoplastic 198 5
Medulloepithelioma
simulating lesion
Uveal melanoma
Data from Shields CL, Kancherla S, Patel J, et al. Clinical survey of 3680 iris
tumors based on patient age at presentation. Ophthalmology 2012;119:407-14 Parasitic cyst
Data from Shields CL, Kancherla S, Patel J, et al. Clinical survey of 3680 iris
tumors based on patient age at presentation. Ophthalmology 2012;119:407-14
segment photography using the slit lamp biomicroscope. In addition,
imaging can be performed with cross-sectional analysis using UBM
Lois et al. studied 17 congenital iris stromal cysts and noted that
or AS OCT.[6] UBM is a high frequency ultrasonographic unit that
those discovered in children under 10 years of age tended to have a
provides acoustic imaging in the range 20-50 megahertz (MHz).
more aggressive course, requiring multiple surgeries and ultimately
Image resolution is approximately 25 microns and to a penetration
with poor visual outcome.[8] Those congenital cysts in teenagers
of 5-6 mm depth. This technique requires the patient to be in a
and adults were more benign in behavior, requiring intervention in
reclined position so that a water bath can be placed on the ocular
about 25% of cases. Shields et al. reviewed a relatively new technique
surface for immersion of the probe. AS OCT, on the other hand, is a
for iris stromal cysts that involves cyst aspiration and wash with
noncontact device that employs a superluminescent diode at 1310
absolute alcohol under viscoelastic protection of the anterior
nanometer wavelength for optical imaging with image resolution
chamber structures.[10] This approach avoids open surgical resection.
of 18 microns and penetration depth of 3-4 mm. AS OCT is a more
They were successful in inducing cyst collapse with sclerosis and
comfortable and convenient imaging modality compared to UBM.
protection of visual acuity in 15 of 16 patients, including children.
In an analysis of 200 consecutive eyes with anterior segment
tumors, mostly in the iris, Bianciotto et al. showed that UBM The IPE cyst is probably more common than previously realized
provided better overall tumor visualization and better resolution now that UBM is commonly detecting this asymptomatic mass.[1,7]
of the posterior margin whereas AS OCT provided better Primary IPE cysts arise on the posterior surface of the iris and were
resolution of the anterior margin.[6] In particular, UBM provides misdiagnosed as ciliary body melanoma in the past due to their
better imaging of pigmented iris tumors as they are optically similar color and configuration. These cysts can be categorized
shadowed on AS OCT. into pupillary margin (3%), mid-zonal (21%), peripheral (73%),
dislodged (3%), and free floating (<1%).[11] Pupillary margin cysts
Tumor categories can be seen without dilation as dark black elongated or collapsed
Cystic lesions of the iris mass at the edge of the pupil. Mid-zonal IPE cysts appear brown to
There are two major categories of primary iris cysts black and fusiform with dome-shaped appearance and emanating
including stromal cysts and pigment epithelial (IPE) cysts[7] on the back of the iris. With dilation, the cyst often everts over
[Table 2 and Figure 1]. The iris stromal cyst can be congenital or the pupillary margin edge onto the anterior iris. This type closely
acquired. The stromal cyst has a characteristic clinical appearance resembles ciliary body melanoma. Peripheral IPE cysts remain
with a smooth surface, lucent mass on or within the iris stroma, hidden in the iridociliary junction and can rarely be visualized
occasionally with fluid-debris level.[8,9] This tumor can enlarge even with wide dilation. They cause an asymptomatic iris stromal
and rupture, leading to secondary iritis, photophobia, pain and bulge and are best detected with UBM or AS OCT as a cystic lesion.
glaucoma. This is especially important in newborn infants as the Dislodged IPE cyst is one that has been presumably free floating
congenital cyst can manifest within the first few weeks of life and and stuck into the anterior chamber angle. Free floating IPE cyst
cause photophobia and buphthalmos. can be found in anterior chamber or in the vitreous and generally

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Shields, et al.: Iris cystic and solid tumors

a b
a b

c d

c d

e f
Figure 1: Iris cysts (a,b) Iris pigment epithelial (IPE) cyst pupillary margin seen
clinically (a) and by optical coherence tomography (b), (c) IPE cyst mid-zonal, (d) IPE
cyst dislodged and in the anterior chamber angle, (e) Iris stromal cyst in an infant, (f) Iris
stromal cyst with hemorrhage in an infant e f
Figure 2: Iris melanocytic tumors (a) Iris nevus (pigmented), (b) Iris nevus
require no treatment. It is important to rule out melanoma and (nonpigmented) causing minor corectopia inferiorly, (c) Iris melanocytoma, (d) Iris
Lisch nodules scattered on the entire iris surface, (e) Iris melanoma involving the
IPE adenoma with each of these types of IPE cysts. anterior chamber angle, (f) Iris melanoma causing corectopia

Iris melanocytic tumors growth into melanoma was detected in 2% of cases. However, by
Iris melanocytic tumors include freckle, nevus, melanocytoma, Kaplan-Meier analysis, growth was detected in 4% by 10 years and
Lisch nodule, and melanoma[1,2] [Figure 2]. A freckle can be single 11% by 20 years. Regarding demographics, patient gender and
or multifocal and rests on the iris stromal surface as a pigmented or race were not predictive of growth. However, age was important
nonpigmented lesion, usually about 1-2 mm in diameter. A nevus and the mean patient age at referral in those that showed growth
shows deeper penetration into the iris stroma, with distortion of was 39 years compared to 52 years in those without growth.
the stroma and often associated with ectropion and corectopia. The authors created a lettering ABCDEF guide to remember the
A melanocytoma is a dark brown to black dome-shaped mass with risk factors predictive of iris nevus growth to melanoma where
little to no ectropion and often with a granular “mound of black A = Age young, B = Blood, C = Clock hour inferior, D = Diffuse
sand” appearance. Occasionally there is minor seeding into the configuration, E = Ectropion and F = Feathery margin[12] [Table 3].
anterior chamber angle or onto the iris stroma. A Lisch nodule These key clinical features help to identify iris melanoma at a time
can be unifocal or multifocal, appears after age 5 years in most when therapy could be life-saving.
cases, has a round, slightly pigmented appearance and is most
often about 1 mm in diameter. Lisch nodule can be a melanocytic Iris melanocytoma is a subset of iris nevus with distinct clinical
marker for neurofibromatosis. These melanocytic tumors are all and histopathologic features. Clinically, this tumor presents as
benign. a deeply pigmented, dark brown domed lesion with a slightly
granular surface.[2,13] The natural course is variable. Most cases
In an analysis of 1611 eyes with iris nevus from a clinical remain stable and require no intervention. Spontaneous necrosis
tertiary referral center, Shields et al. found that the mean age at with resultant pigment dispersion and secondary glaucoma can
referral was 51 years and the mean tumor basal diameter was occur and transformation to melanoma is rarely encountered.
3 millimeters (mm).[12] Keep in mind, most of these iris nevi In an analysis of 47 eyes with iris melanocytoma, the mean
were considered to have suspicious features for melanoma and patient age at presentation was 37 years. The mass appeared
hence were referred for evaluation to an ocular oncology center. nodular (85%) or diffuse (15%) and was heavily pigmented in
The nevus was found in male (39%) or female (61%) patients. nearly every case.[13] The melanocytoma was located in the
The patient race was Caucasian (97%), African American (2%), inferior (45%) or lateral (38%) quadrants most often. The tumor
Hispanic (<1%), Asian(<1%), or other (each < 1%). The iris color was a mean size of 3 mm diameter and thickness of 2 mm. Other
was blue (51%), green (21%), or brown (27%). In this analysis, features included heterochromia (13%), satellite tumors (26%),

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Shields, et al.: Iris cystic and solid tumors

Table 3: The ABCDEF guide for factors predictive extending posteriorly into ciliary body or choroid, T3 with
of transformation of iris nevus to iris melanoma additional scleral extension, and T4 with additional extrascleral
Letter Feature Outcome Hazard extension.[21] In a collaborative report on 131 eyes with iris
Growth into Growth into ratio melanoma, Khan and coworkers found 56% of tumors as T1,
melanoma melanoma 34% of tumors as T2, 2% of tumors as T3 and 1% of tumors as
present absent T4.[22] Survival without metastasis was 100% for T1, 90% for T2
n=27 (%) n=1584 (%)
and 50% for T3 and T4.
A Age (<40 years) 3 97 3
B Blood 25 75 9
C Clock hour inferiorly 2 98 9
Iris non-melanocytic tumors
D Diffuse configuration 17 83 14 In a large analysis of 3680 iris tumors, non-melanocytic lesions
E Ectropion 4 96 4 were found in 402 (11%) cases.[1] The non-melanocytic tumors
F Feathery margin 4 96 3 included categories of choristomatous (<1%), vascular (2%),
Data from Shields CL, Kaliki S, Hutchinson A, et al. Iris nevus growth fibrous (<1%), neural (<1%), myogenic (<1%), epithelial (1%),
into melanoma: Analysis of 1611 consecutive eyes. The ABCDEF guide. xanthomatous/xanthogranulomatous (<1%), metastasis (2%),
Ophthalmology 2013;120:766-72
lymphoid (<1%), leukemic (<1%), secondary (<1%), and
non-neoplastic simulators (5%) [Table 1 and Figure 3].
ectropion (6%). The tumor had shed seeds into the anterior
chamber angle in 26% for a mean extent of 8 clock hours. By Of 57 vascular iris tumors, the most common included racemose
Kaplan-Meier analysis at 10 years follow-up, new tumor seeds hemangioma (n = 37) and cavernous hemangioma (n = 7).[1]
developed in 63%, secondary glaucoma in 11%, and mild growth Vascular iris tumors were more common in mid-adult and senior
in 48%.[13] The growth was attributed slow benign enlargement in adults (n = 43) compared to children and young adults (n = 14).
all cases and there was no instance of transformation to melanoma Other studies have described various iris vascular tumors.[23-25]
in that series. Shields et al. reviewed iris vascular tumors in an analysis of
45 cases and found several iris vascular tumors including racemose
Iris melanoma is a malignant iris tumor with risk for metastasis hemangioma, cavernous hemangioma, capillary hemangioma,
and death.[14-20] In a large analysis of 8033 eyes with melanoma, varix and microhemangiomatosis.[25] Most vascular tumors
Shields et al. found 4% of iris origin and 96% of choroidal or occurred in adults at a mean age of 55 years. They noted that
ciliary body origin.[14] A subsequent series of 317 patients with transient hyphema was the main complication, present in 30%
iris melanoma revealed this tumor to occur in children (8%), of eyes.[25]
mid-adults (59%), and older adults (33%).[15] Iris melanoma
showed features of location in the inferior quadrant (45%) most Of the 67 metastatic iris tumors, the malignancy arose from
often, mean basal diameter of 6.2 mm and mean thickness of carcinoma of the breast (n = 21), lung (n = 19), kidney (n = 3),
2.3 mm, partially or completely pigmented in 90%, and features and skin [melanoma] (n = 9).[1] Metastatic iris tumors were more
of corectopia (45%), ectropion uveae (24%), hyphema (3%), common in mid-adult and senior adults (n = 61) compared to
glaucoma (35%), angle seeding (28%) for mean 2 clock hours, children and young adults (n = 6). In a publication on 40 patients
and extraocular extension (3%).[15] The mean intraocular pressure with metastatic tumors to the iris, the authors noted that all
was 20 mm Hg, but it ranged from 17 to 80 mm Hg. There was metastases were unilateral and secondary glaucoma was present
no age-related differences in race, sex, tumor location, or tumor in 38%. In 32% of cases there was no history of previous cancer
thickness. There was an age-related difference in tumor basal and systemic evaluation disclosed the primary site in the lung,
diameter, intraocular pressure and tumor seeding based on age, colon, breast, kidney and skin.
with increasing features with age in years. By Kaplan-Meier
analysis, iris melanoma lead to metastasis in 5% at 5 years, 9% Another subset of non-melanocytic iris tumors includes solid
at 10 years, and 11% at 20 years.[15] By multivariate analysis, epithelial tumors. In the large analysis on 3680 cases, there
the main factors predictive of metastasis included extraocular were 35 cases of iris epithelial solid tumors including IPE
extension and elevated intraocular pressure.[15] Treatment of iris adenoma (n = 31), medulloepithelioma of iris (n = 3), and IPE
melanoma includes surgical resection if the tumor is confined to adenocarcinoma (n = 1).[1] The medulloepitheliomas generally
3-4 clock hours and without seeding. Plaque radiotherapy can be had a component in the non-pigmented ciliary epithelium and
employed if the tumor is large and with seeding, keeping the eye arose in children whereas the adenoma arose in mid-adults or
intact without entering the anterior chamber.[20] Enucleation is senior adults (84%). The single case of adenocarcinoma arose
advised if there is uncontrollable secondary glaucoma. in young adult. Other series on iris pigment epithelial adenoma
and adenocarcinoma have provided descriptive details of this
The American Joint Committee on Cancer (AJCC) staging tumor and its notably lack of metastatic disease, despite showing
manual, 7th edition, provides a detailed classification for anterior aggressive malignant cytology.[26]
(iris) and posterior (ciliary body and choroid) uveal melanoma
for prognostication. In this classification, iris melanoma is All xanthoma/xanthogranuloma (n = 16, <1%) of the iris, in this
classified as T1 with tumor limited to the iris, T2, with tumor large analysis, occurred in children.[1] From a different perspective,

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Shields, et al.: Iris cystic and solid tumors

of the total 446 iris tumors in children, the most common and heterochromia (n = 19)[1] [Table 4]. In children, there were
diagnoses included iris nevus (24%), IPE cyst (28%), iris stromal 49 non-neoplastic lesions simulating iris tumor and these included
cyst (7%), juvenile xanthogranuloma (4%) and melanoma (8%). most often iris coloboma (n = 17) and congenital heterochromia
(n = 10), compared to the 113 simulators in mid- and senior adults
Of the 198 non-neoplastic lesions simulating iris tumors, the most that proved to be iris atrophy (n = 33), iridocorneal endothelial
common diagnoses were iridocorneal endothelial syndrome (n = 50), syndrome (n = 32) and foreign body (n = 15). In a separate analysis
iris atrophy (n = 42), foreign body (n = 20), coloboma (n = 20) of 71 cases of iridocorneal endothelial syndrome simulating iris
melanoma, features that differentiated this degenerative condition
Table 4: Non-neoplastic lesions of the iris that from iris melanoma included corneal guttatae, multidirectional
simulate an iris tumor corectopia, polycoria, broad peripheral anterior synechiae,
iris pigment epithelial defects, and lack of elevated mass with
Diagnosis Number (%)
(N=198 tumors) iridocorneal endothelial syndrome[28] [Figure 4].
Iridocorneal endothelial syndrome 50 (25)
Iris atrophy 42 (21) Discussion
Foreign body 20 (10)
Coloboma 20 (10)
In the past, most series on iris tumors have emanated from pathology
Congenital heterochromia 19 (10)
Iris granuloma 13 (7) laboratories. In 1958, Duke and Dunn reported on 43 cases
Pigment dispersion 11 (6) of iris tumors in a pathology laboratory and found malignant
Iris depigmentation 6 (3) melanoma (n = 28), “benign melanoma” [nevus, melanocytoma]
Congenital ectropion irides 6 (3) (n = 13), leiomyoma (n = 1) and uncertain (n = 1).[3] In 1963,
Iridoschisis 4 (2) Ashton published 145 iris tumors from a pathology department
Iridodialysis 3 (2)
and found iris melanoma (n = 105), leiomyoma (n = 21),
Congenital iris hypopigmentation 2 (1)
Candidiasis 1 (<1)
nevus (n = 10), freckle (n = 4), angioma (n = 3) and IPE
Unknown 3 (2) tumors (n = 2).[4] In 1964, Heath reported on 232 iris tumors
Data from Shields CL, Kancherla S, Patel J, et al. Clinical survey of 3680 iris from a pathology laboratory including melanoma (n = 113),
tumors based on patient age at presentation. Ophthalmology 2012;119:407-14 secondary invasion into iris (n = 70), leiomyoma (n = 5),
metastatic tumors (n = 6), vascular tumors (n = 6) and other
rare conditions.[5] In 2012, Shields et al. reported the first
comprehensive clinic-based series of iris tumors in 3680 cases.[1]
Much of the information in this review was extracted from this
report. They categorized iris tumors [Table 1] and provided
organization and illustrations to recognize the various types of
iris lesions. In addition, they specifically evaluated the age at
diagnosis of the various iris tumors and found that the median
age (years) at diagnosis included cystic (39), melanocytic (52),
a b
choristomatous (0.7), vascular (56), fibrous (53), neural (8),
myogenic (42), epithelial (63), xanthomatous (1.9), metastasis
(60), lymphoid (57), leukemic (25.5), secondary (59) and
non-neoplastic simulators (49).[1] Overall, in this series from a
tertiary referral ocular oncology center, the three most common
specific diagnoses (children, young adult, mid-adult, senior

c d

e f a b
Figure 3: Iris non-melanocytic tumors (a,b) Iris vascular tumor diagnosed as racemose Figure 4: Non-neoplastic tumors simulating iris tumors (a) Iridocorneal endothelial
hemangioma, barely seen clinically (a) as ectatic vessel, but more obvious on syndrome with temporally directed ectropion, corectopia contracted iris and broad
fluorescein angiography (b) as tortuous dilated vessel (c) Iris juvenile xanthogranuloma peripheral anterior synechiae, (b) Iridocorneal endothelial syndrome with broad
in a one-year-old child, (d) Iris lymphoid tumor, (e) Iris metastasis from breast cancer, peripheral anterior synechiae inferotemporally and iris adherence to endothelium with
(f) Iris metastasis from remote cancer iris stromal tear, allowing visualization of the underlying iris pigment epithelium

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Shields, et al.: Iris cystic and solid tumors

adult) were nevus (25%, 36%, 47%, 47%), IPE cyst (28%, 30%, melanocytoma: Clinical features and natural course in 47 cases. Am J
Ophthalmol 2005;139:468-75.
15%, 14%) and melanoma (8%, 16%, 20%, 19%).[1]
14. Shields CL, Kaliki S, Furuta M, Mashayekhi A, Shields JA. Clinical
spectrum and prognosis of uveal melanoma based on age at presentation
In summary, in this report we provide an overview of the clinical in 8033 cases. Retina 2012;32:1363-72.
features and frequency of iris tumors. Iris tumors are broadly 15. Shields CL, Kaliki S, Shah SU, Luo W, Furuta M, Shields JA. Iris melanoma
classified into cystic or solid lesions. Cystic lesions are generally features and prognosis in children and adults in 317 patients. The 2011
Leonard Apt Lecture. J AAPOS 2012;16:10-6.
benign. Solid lesions are most often melanocytic. Iris nevus is the
16. van Klink F, de Keizer RJ, Jager MJ, Kakebeeke-Kemme HM. Iris nevi and
most common solid iris tumor in all age groups. Risk factors for melanomas: A clinical follow-up study. Doc Ophthalmol 1992;82:49-55.
transformation of nevus into melanoma can be recalled using the 17. Shields CL, Shields JA, Materin M, Gershenbaum E, Singh AD, Smith A.
ABCDEF guide. Iris melanoma: Risk factors for metastasis in 169 consecutive patients.
Ophthalmology 2001;108:172-8.
18. Demirci H, Shields CL, Shields JA, Eagle RC Jr, Honavar SG. Diffuse iris
References melanoma: A report of 25 cases. Ophthalmology 2002;109:1553-60.
19. Henderson E, Margo CE. Iris melanoma. Arch Pathol Lab Med
1. Shields CL, Kancherla S, Patel J, Vijayvargiya P, Suriano MM, Kolbus E, 2008;132:268-72.
et al. Clinical survey of 3680 iris tumors based on patient age at 20. Shields CL, Shah S, Bianciotto CG, Emerich JE, Komarnicky L, Shields JA.
presentation. Ophthalmology 2012;119:407-14. Iris melanoma management with Iodine-125 plaque radiotherapy in
2. Shields JA, Shields CL. Intraocular Tumors. An Atlas and Textbook. 2nd ed. 144 patients: Impact of melanoma-related glaucoma on outcomes.
Philadelphia: Lippincott Williams and Wilkins; 2008. p. 3-58. Ophthalmology 2013;120:55-61
3. Duke JR, Dunn SN. Primary tumors of the iris. Arch Ophthalmol 21. Edge SB, Byrd DR, Compton CC, Fritz AG, Greene FL, Trotti A, et al.
1958;59:204-14. AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer; 2009. p.
4. Ashton N. Primary tumours of the iris. Br J Ophthalmol 1964;48:650-68. 547-59.
5. Heath P. Tumors of the iris. Classification and clinical follow-up. Trans Am 22. Khan S, Finger PT, Yu GP, Razzaq L, Jager MJ, de Keizer RJ, et al.
Ophthalmol Soc 1964;62:51-82. Clinical and pathologic characteristics of biopsy-proven iris melanoma.
6. Bianciotto C, Shields CL, Guzman JM, Romanelli-Gobbi M, Mazzuca D Jr, A multicenter international study. Arch Ophthalmol 2012;130:57-64.
Green WR, et al. Assessment of anterior segment tumors with ultrasound 23. Shields JA, Streicher TF, Spirkova JH, Stubna M, Shields CL. Arteriovenous
biomicroscopy versus anterior segment optical coherence tomography in malformation of the iris in 14 cases. Arch Ophthalmol 2006;124:370-5.
200 cases. Ophthalmology 2011;118:1297-302. 24. Broaddus E, Lystad LD, Schonfield L, Singh AD. Iris varix report of a case
7. Shields JA. Primary cysts of the iris. Trans Am Ophthalmol Soc and review of iris vascular anomalies. Surv Ophthalmol 2009;54:118-27.
1981;79:771-809. 25. Shields JA, Bianciotto CG, Kligman B, Shields CL. Vascular tumors of
8. Lois N, Shields CL, Shields JA, Mercado G. Primary iris stromal cysts: the iris. A review of 45 patients. The 2009 Helen Keller Lecture. Arch
A report of 17 cases. Ophthalmology 1998;105:1317-22. Ophthalmol 2010;128:1107-12.
9. Shields JA, Shields CL, Lois N, Mercado G. Iris cysts in children: 26. Shields JA, Shields CL, Kiratli H, De Potter P. Metastatic tumors to the iris
Classification, incidence and management. The 1998 Torrence A Makley in 40 patients. Am J Ophthalmol 1995;119:422-30.
Jr. Lecture. Br J Ophthalmol 1999,83:334-8. 27. Shields JA, Shields CL, Mercado G, Gündüz K, Eagle RC Jr. Adenoma
10. Shields CL, Arepalli S, Lally SE, Lally EB, Shields JA. Iris stromal cyst of the iris pigment epithelium: A report of 20 cases. Arch Ophthalmol
management with alcohol-induced sclerosis in 16 patients. JAMA 1999;117:736-41.
Ophthalmol 2014. [In press]. 28. Shields CL, Shields MV, Viloria V, Pearlstein H, Say EA, Shields JA.
11. Lois N, Shields CL, Shields JA, Mercado G. Primary cysts of the iris Iridocorneal endothelial syndrome masquerading as iris melanoma in
pigment epithelium: Clinical features and natural course in 234 patients. 71 cases. Arch Ophthalmol 2011;129:1023-9.
Ophthalmology 1998;105:1879-85.
Cite this article as: Shields CL, Shields PW, Manalac J, Jumroendararasame
12. Shields CL, Kaliki S, Hutchinson A, Nickerson S, Patel J, Kancherla S,
C, Shields JA. Review of cystic and solid tumors of the iris. Oman J Ophthalmol
et al. Iris nevus growth into melanoma: Analysis of 1611 consecutive eyes.
2013;6:159-64.
The ABCDEF guide. Ophthalmology 2013;120:766-72.
Source of Support: Nil, Conflict of Interest: None declared.
13. Demirci H, Mashayekhi A, Shields CL, Eagle RC Jr, Shields JA. Iris

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