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Clinicopathologic Reports, Case Reports, and Small Case Series
February 2002

Pigmented Medulloepithelioma of the Ciliary Body

Arch Ophthalmol. 2002;120(2):207-210. doi:

A 3-year-old girl developed leukocoria in her right eye and was found to have a fibrovascular cyclitic membrane and a pigmented ciliary body mass. Following removal of the mass by iridocyclochoroidectomy and localized vitrectomy, it proved histopathologically to be a malignant medulloepithelioma with cytoplasmic melanin pigment. Five months later, tumor recurrence necessitated enucleation. Although ciliary body medulloepithelioma is usually an amelanotic, fleshy mass with an intralesional cystic component, it can occur as a pigmented solid tumor that may resemble a melanoma or a neoplasm of the pigment epithelium.

Intraocular medulloepithelioma is a well-known but uncommon pediatric neoplasm that usually occurs in the ciliary body.14 This embryonic tumor is believed to arise from the primitive medullary epithelium that composes the anterior lip of the optic cup.5 It usually occurs on the ciliary body of a young child as a fleshy gray or pink mass, often with clinically evident cysts. Although small amounts of pigment occasionally can be observed pathologically in the cytoplasm of medulloepithelioma cells, such pigmentation is not observed clinically.4 A pigmented medulloepithelioma in the central nervous system has been reported,6 but we are unaware of a clinically pigmented medulloepithelioma of the ciliary body. We report a clinicopathologic correlation of a malignant medulloepithelioma of the ciliary body that was intensely pigmented clinically and histopathologically.

Report of a Case

In July 1997, a 3-year-old white girl with a normal birth and medical history was noted to have intermittent exotropia and a white pupillary reflex in her right eye. Examination revealed a ciliary body mass and the patient was referred to the Oncology Service at Wills Eye Hospital. The right eye had a visual acuity of light perception and an intraocular pressure of 21 mm Hg. There was a deeply pigmented mass in the ciliary body inferotemporally. A membrane of dense fibrovascular tissue emanated from the area of the tumor and surrounded the equatorial region of the lens (Figure 1). A moderately dense nuclear and posterior subcapsular cataract precluded a clear view of the fundus. A coincidental irregular iris nevus was present from the 9- to- 10-o'clock position location in the iris stroma (Figure 1). B-scan ultrasonography showed a pedunculated mass that had medium to high echogenicity, arose from the ciliary body, and measured 9 mm in diameter and 7 mm in thickness. The left eye had a visual acuity of 20/20 and was entirely normal.

Figure 1.
A pigmented ciliary body mass
and vascularized cyclitic membrane is present in the ciliary body inferotemporally.
There is an irregular iris nevus superior to the lesion.

A pigmented ciliary body mass and vascularized cyclitic membrane is present in the ciliary body inferotemporally. There is an irregular iris nevus superior to the lesion.

Our clinical differential diagnosis included neoplasm of the ciliary body pigment epithelium, medulloepithelioma, melanocytoma, and melanoma. The parents were offered the options of enucleation, fine-needle aspiration biopsy, or local resection. They elected to have the tumor removed by local resection. A partial lamellar iridocyclochoroidectomy and localized vitrectomy were performed7 and it appeared that the entire mass was removed intact along with fragments of lens to which the tumor was adherent. Moderate bleeding from the cyclitic membrane occurred during tumor removal.

In September 1997, the patient was found to have a tractional retinal detachment in the affected eye and underwent a pars plana vitrectomy, lensectomy, and scleral buckling procedure with successful retinal reattachment. At the time of that surgery, however, a light tan mass was observed just superior to the area of the original tumor (Figure 2). The patient was referred to the Oncology Service and enucleation was performed. There is no local recurrence or metastasis 3 years postenucleation.

Figure 2.
Standard slitlamp photograph through
dilated pupil showing recurrence of the tumor which appears as a dome-shaped,
light tan mass in the ciliary body.

Standard slitlamp photograph through dilated pupil showing recurrence of the tumor which appears as a dome-shaped, light tan mass in the ciliary body.

Pathologic Findings

On gross examination, the original tumor removed by iridocyclochoroidectomy measured 11 × 8 × 6 mm. Microscopically it consisted of a scleral base, a pigmented superficial portion, and a nonpigmented deeper portion (Figure 3). The area that appeared pigmented grossly was composed microscopically of cords and tubules of well-polarized epithelium that was partially pigmented (Figure 3). The area was surrounded by a fibrous matrix containing spindle cells. Single, intensely pigmented cells, probably melanophages, were admixed with the neuroepithelial tubules (Figure 4). Corresponding to the less pigmented peripheral area seen grossly were spindle cells and tubules of nonpigmented epithelium set in a matrix of mucopolysaccharide (Figure 5) that stained positive for colloidal iron (Figure 6) and that was partially sensitive to hyaluronidase digestion. The pigment showed positive staining with the Fontana-Masson stain, confirming that it was melanin. Areas of the tumor contained a sarcomatoid arrangement of the cells, but no convincing skeletal/ muscle or cartilage was identified in the available sections. The tumor incorporated and encompassed the equatorial portion of fragmented lens tissue. The stroma of the pars plana was infiltrated by pigmented tumor cells. A few mitotic figures were present and the nuclei appeared pleomorphic and hyperchromatic. Tumor cells were present very close to one of the surgical margins. The final diagnosis was pigmented medulloepithelioma. It was classified as malignant on the basis of cytologic features, mitotic activity, and focal invasion of the ciliary body stroma.

Figure 3.
Histopathologic features of the
resected tumor are the pigmented neuroepithelial tubules in the superficial
area of the tumor (top right) and the nonpigmented sarcomatoid area (bottom
left) (hematoxylin-eosin, original magnification ×50).

Histopathologic features of the resected tumor are the pigmented neuroepithelial tubules in the superficial area of the tumor (top right) and the nonpigmented sarcomatoid area (bottom left) (hematoxylin-eosin, original magnification ×50).

Figure 4.
Most of the pigmented portion
of the tumor comprisesf cords and tubules of neuroepithelial cords and tubules
that contain cytoplasmic pigment. Note the rosettelike formation (hematoxylin-eosin,
original magnification ×50).

Most of the pigmented portion of the tumor comprisesf cords and tubules of neuroepithelial cords and tubules that contain cytoplasmic pigment. Note the rosettelike formation (hematoxylin-eosin, original magnification ×50).

Figure 5.
Pool of lucent material surrounding
a tubule of polarized neuroepithelium in the nonpigmented area of the tumor
(hematoxylin-eosin, original magnification ×100).

Pool of lucent material surrounding a tubule of polarized neuroepithelium in the nonpigmented area of the tumor (hematoxylin-eosin, original magnification ×100).

Figure 6.
Colloidal iron stain discloses
large amounts of mucopolysaccharide surrounding the neuroepithelial elements
(colloidal iron, original magnification ×100).

Colloidal iron stain discloses large amounts of mucopolysaccharide surrounding the neuroepithelial elements (colloidal iron, original magnification ×100).

Histopathologic study of the enucleated eye revealed the expected changes following removal of a ciliary body tumor and subsequent vitrectomy and retinal detachment surgery. The most noteworthy histopathologic finding was a nodule in the ciliary body area immediately superior to the area of prior tumor resection. Most of the nodule consisted of a florid proliferation of spindle-shaped fibroblasts that appeared to be organizing areas of degenerated blood. The base of the nodule was composed of cords, bands, and islands of pigmented cells that resembled those seen in the previously resected pigmented medulloepithelioma. No heteroplastic elements were identified. The main diagnosis was recurrent or residual pigmented medulloepithelioma.

Comment

Intraocular medulloepithelioma is a rare embryonic neoplasm that usually is diagnosed in the first decade of life as a ciliary body mass.14 It can be classified as benign or malignant and as teratoid or nonteratoid.5 Histopathologic criteria for malignancy include the presence of undifferentiated areas that resemble retinoblastoma or sarcoma and local invasiveness. Metastasis is very rare and usually occurs in cases with extraocular extension. In 2 reported series the tumor was classified as malignant in 66%1 and 90%.4 The nonteratoid medulloepithelioma is a pure neoplastic proliferation of cells that resembles the medullary epithelium without heteroplastic elements, whereas the teratoid variant contains heteroplastic elements like hyaline cartilage, rhabdomyoblasts, striated muscle, and neural tissue resembling brain. The tumor in our patient was a malignant teratoid medulloepithelioma.

Clinically, ciliary body medulloepithelioma is usually a fleshy pink lesion. Large cystic spaces that contain vitreouslike material may be present. Often there is a fibrovascular cyclitic membrane containing cords of tumor cells.4 The medulloepithelioma in our patient was unusual in that it was deeply pigmented clinically and histopathologically and lacked a cystic component. A pigmented medulloepithelioma of the central nervous system has been reported as a tumor in the fourth ventricle in a 9-year-old boy.6 We are aware of one other case of clinically pigmented medulloepithelioma of the ciliary body. It occurred in an 18-year-old African woman who had a black epibulbar mass and expulsive hemorrhage.8 Hence, the appearance of that ciliary body medulloepthelioma was quite different from the one seen in our case. Although most medulloepitheliomas lack pigment clinically, a few melanin granules are sometimes observed microscopically in ciliary body medulloepitheliomas.4

Our case showed findings consistent with other reported malignant medulloepitheliomas, except that the cytoplasm of many of the neuroectodermal cells contained pigment. We do not believe that the tumor arose from the pigment epithelium because pigment epithelial tumors do not produce hyaluronic acid, which was present in our patient's tumor. Concerning the origin of the melanin pigment in our case, it is known that pigmentation can be present in other tumors of neuroectodermal origin, including ependymoma, cerebellar medulloblastoma, schwannoma, meningioma, and pigmented neuroectodermal tumor of infancy.6 Melanin pigment is also a transient feature in the fetal pineal gland and is sometimes present in pinealoblastomas.6 Therefore, it is not surprising that melanin pigmentation may also occur in intraocular medulloepithelioma, which is also a neoplasm of neuroectodermal derivation.

The differential diagnosis of a pigmented medulloepithelioma includes ciliary body melanoma, neoplasm of the pigment epithelium of the ciliary body, melanocytoma, and iridociliary cyst. Ciliary body melanoma can occur in children9,10 and can appear very similar to the tumor in our case. However, the cyclitic membrane seen in our patient would be unexpected with a ciliary body melanoma. Adenoma of the pigment epithelium of the ciliary body occurs mostly in adult patients,11 but it can occasionally occur in children.12 The adenoma of the ciliary body pigment epithelium reported in a child by Campochiaro and associates12 was remarkably similar to our case in that it was a pigmented mass associated with a cyclitic membrane. Iridociliary cysts can appear pigmented, but they do not produce a cyclitic membrane and secondary cataract.13,14

Our case exemplifies the difficulties encountered in local resection of a ciliary body medulloepithelioma. Local surgical removal of this tumor is frequently complicated by extensive bleeding from the fibrovascular neoplastic cyclitic membrane. Recurrence is frequent because the tumor often grows as a thin sheet on the surface of ocular structures, which may be inapparent at the time of local tumor removal.4,15 Neoplastic cells can be left behind in the cyclitic membrane and enucleation often becomes necessary because of residual or recurrent tumor.4 Iridocyclectomy can be successful only for relatively small tumors that are not associated with an extensive neoplastic cyclitic membrane. Interestingly, this tendency for recurrence contrasts to acquired adenomas of the nonpigmented and pigmented epithelium of the ciliary body, which can often be completely removed by iridocyclectomy.16 In summary, we report the clinical and histopathologic features of a malignant medulloepithelioma of the ciliary body that was clinically and histopathologically pigmented. This unique variation of a rare neoplasm should be included in the differential diagnosis of pigmented lesions of the ciliary body.

This study was by supported the Eye Tumor Research Foundation, Philadelphia, Pa (Drs C. L. Shields and J. A. Shields), the Award of Merit in Retina Research, Houston Tex (Dr J. A. Shields), the Macula Foundation, New York, NY (Dr C. L. Shields), and the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital, Philadelphia (Dr Eagle).

This study was presented at the combined meeting of the Armed Forces Institute of Pathology Ophthalmic Alumni Society, Theobald Society, and Hogan Society, Washington, DC, April 20, 2001.

A. K. Samad, MD, and Edward J. Doherty, OD, assisted in the care of the patient. Lorenz E. Zimmerman, MD, and Ian W. McLean, MD, reviewed the histopathologic sections and concurred with the diagnosis.

Reprints not available from the authors.

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