A 77-year-old woman underwent iridocyclectomy for a progressively enlarging
black mass that apparently originated in the ciliary body and secondarily
invaded the iris of her pseudophakic left eye. At the time of surgery, the
tumor was adherent to the intraocular lens, which was removed along with the
mass. Histopathologically, the tumor was composed of islands and cords of
benign pigment epithelial cells that were separated by vascularized fibrous
connective tissue. The diagnosis was adenoma of the ciliary body pigment epithelium
with secondary invasion of the iris. This case underscores the fact that benign
intraocular tumors can show dramatic enlargement without undergoing malignant
The pigment epithelial layers of the eye often undergo reactive hyperplasia
but true neoplasia of ocular pigment epithelia is rare.1- 4
On occasion, however, true neoplasms can develop from the pigment epithelia
of the iris, ciliary body, and retina.5- 7
Tumors of the pigment epithelium generally are benign or low-grade malignancies
and have no known tendency to metastasize. However, they can grow slowly and
can be locally invasive.5- 7
We report a clinicopathologic correlation of an adenoma of the ciliary body
pigment epithelium that secondarily invaded the iris and developed into a
sizable intraocular mass.
In 1993, a 70-year-old woman was found on routine ocular examination
to have an early cataract in her left eye. At that time, her irides were normal
and no pigmented lesions were noted. In 1995, the cataract had progressed
and a small pigmented lesion was first noticed in her left iris. This was
interpreted as a melanocytic nevus. In 1996, the cataract had shown further
progression and successful cataract extraction with placement of a posterior
chamber intraocular lens was performed. Two years later, the iris lesion had
shown no convincing change, but suspicion of possible melanoma prompted referral
to the oncology service.
At the time of our initial evaluation in 1997, the corrected visual
acuity was 20/20 OD (phakic eye) and 20/20 OS (pseudophakic eye). Intraocular
pressure readings were normal. In the nasal aspect of the left iris was a
black lesion that spared the pupillary margin and extended to involve the
base of the iris between the 8:30- and 10-o'clock meridians (Figure 1). The mass appeared to be pushing anteriorly through the
iris stroma from the ciliary body but it did not seem to arise from or infiltrate
the stroma. Gonioscopy showed that the lesion obscured a view of the angle
structures and there was fine pigment dusting in the angle inferiorly. Ultrasound
biomicroscopy results revealed ciliary body thickening by the mass and transillumination
disclosed a shadow that extended across the pars plicata for 1 mm into the
pars plana. The favored diagnosis was adenoma of the ciliary body pigment
epithelium with secondary extension through the iris. Because of her age and
good visual acuity, it was elected to continue to follow the patient conservatively.
In 1997, the iris component of
the lesion appeared as a black mass in the nasal aspect of the iris.
In 1998, the lesion was first noted to be displacing the intraocular
lens. In 1999, the patient's visual acuity had decreased to 20/40 OS and the
intraocular lens appeared to be covered by fibrous tissue in the quadrant
of the tumor. An episcleral sentinel blood vessel had developed over the tumor
and the angle pigmentation inferiorly had become denser. By 2000, the iris
component of the lesion had become larger (Figure 2) and ultrasonography showed the ciliary body component
of the lesion to be 6 mm thick. With transillumination, the lesion measured
7 × 7 mm in diameter and extended more posteriorly into the pars plana,
almost reaching the ora serrata.
In 2000, the iris component of
the lesion was slightly larger. Transillumination and ultrasonography results
revealed more pronounced ciliary body involvement posteriorly. The pupil has
been dilated pharmacologically.
Because of the progressive enlargement, the mass was resected by a partial
Firm adherence of the mass to the intraocular lens made them inseparable,
necessitating removal of the lens along with the tumor. Postoperatively, there
was diffuse vitreous hemorrhage, which slowly resolved during 3 months without
the need for a vitrectomy.
Grossly, the black lesion had clear scleral margins. The mass partially
encompassed the lens capsular bag, which contained an intraocular lens (Figure 3). Microscopically, the specimen
consisted of a lamella of sclera on which rested a pigmented tumor that was
passing through the thinned iris (Figure 4). The mass was composed of nests, cords, and islands of intensely
pigmented epithelial cells that were separated by prominent septa of vascularized
fibrous connective tissue (Figure 5).
The cords were solid without evidence of duct formation. The melanin granules
in the cytoplasm were large and generally spherical in configuration, resembling
those seen in the normal pigment epithelium. Bleached sections showed that
the tumor nuclei were generally round or oval with relatively well-dispersed
chromatin and small to mildly conspicuous nucleoli (Figure 6). The nuclei were only mildly pleomorphic and only 2 mitotic
figures were counted in 40 high-power fields. The posterior surgical margin
was free of tumor. Fibrous tissue encompassed a groove in the tumor that contained
lens cortical and capsular remnants and a space that was occupied by the intraocular
lens haptic in vivo. This was surrounded by capsular fibrosis. There was mild
invasion of the ciliary body stroma by the tumor. The final diagnosis was
adenoma of the ciliary body pigment epithelium with secondary iris invasion.
Gross appearance of the resected
lesion shows the black mass (to the left) with a sclera base (bottom). There
is a firm attachment of the black mass to the intraocular lens (to the left).
Pigmented mass with a section
of normal iris adjacent to the lesion (hematoxylin-eosin; original magnification
The tumor is composed of islands
and cords of deeply pigmented epithelial cells with connective tissue stroma
(hematoxylin-eosin; original magnification ×200).
Bleached section showing bland
cells with uniform nuclei (bleached, hematoxylin-eosin; original magnification
Tumors of the ciliary body pigment epithelium are rare.6,9- 13
Until recently, they were often misdiagnosed clinically as malignant melanoma.
In our case, the diagnosis was suspected clinically because of the black color
of the lesion. Tumors of the pigment epithelium traditionally have been considered
to be relatively dormant but a recent study revealed that those in the ciliary
body can cause vitreous hemorrhage, subluxation of the lens, and secondary
cataract,6 and that those of the retinal
pigment epithelium can be locally invasive and cause exudative retinal detachment.7 Conversely, tumors of the iris pigment epithelium
tend to be more stationary and are less likely to exhibit local invasion.
In a clinical series of 20 consecutive cases of adenoma of the iris pigment
epithelium, only 2 required surgical removal and 18 remained stationary during
the course of follow up.5 The tumor in our
patient was initially suspected be an iris lesion but detailed examination
suggested that it probably originated in the pigment epithelium of the ciliary
body. Documented growth of the tumor prompted removal and the diagnosis was
A remarkable histopathologic feature of the tumor reported here is that
it was composed predominantly of solid cords and tubules of tumor cells. These
findings are more consistent with reported cases of adenomas of the iris pigment
epithelium.5 In contrast, tumors that arise
from the ciliary body pigment epithelium are usually characterized by numerous
clear vacuoles and have less connective tissue stroma.6
The tumor in our patient lacked such vacuoles. From a clinical standpoint,
however, the tumor appeared to originate in the ciliary body and to secondarily
invade the iris.
The tumor reported here showed progressive enlargement, secondarily
invaded the iris, induced the development of a sentinel blood vessel, and
displaced and partially adhered to the intraocular lens. Physicians should
be aware that progressive growth of a ciliary body or iris mass does not necessarily
imply malignant transformation. Ancillary measures such as gonioscopy, transillumination,
and ultrasound biomicroscopy are often necessary to determine the size and
extent of a ciliary body mass. Recognition of the characteristic features
of adenoma of the ciliary body pigment epithelium should facilitate the diagnosis
of this unusual tumor.
This research was supported by the Eye Tumor Research Foundation, Philadelphia,
the Award of Merit in Retina Research, Houston, Tex (Dr J. A. Shields), the
Macula Foundation, New York, NY (Dr C. L. Shields), and by the Noel T. and
Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital, Philadelphia
We thank Anthony LaTessa, MD, and Richard A. Frio, OD, for their help
in the management of the patient.
Corresponding author and reprints: Jerry A. Shields, MD, Oncology
Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107.
Shields JA, Eagle RC, Shields CL, Singh AD, Torrisi PF. Progressive Growth of Benign Adenoma of the Pigment Epithelium of the Ciliary Body. Arch Ophthalmol. 2001;119(12):1859-1861. doi: