[Skip to Content]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address 54.211.168.204. Please contact the publisher to request reinstatement.
[Skip to Content Landing]
Case Reports and Small Case Series
March 2001

Presumed Bilateral Medulloepithelioma

Arch Ophthalmol. 2001;119(3):449-450. doi:

Medulloepitheliomas are rare embryonic tumors usually arising from the ciliary body.1 None of the rare series published in the literature describe bilateral ciliary body involvement.13 We describe the case of a 7-year-old boy who was diagnosed with bilateral ciliary body tumors, in whom the clinical features were highly suggestive of medulloepithelioma. Histological examination of the excisional biopsy specimen from the right eye confirmed the diagnosis of medulloepithelioma.

Report of a Case

A 7-year-old boy was referred to our department with bilateral ciliary body tumors. His ocular history included an episode of a red and painful right eye 1 year previously. Excision of a ciliary body mass from his right eye revealed histological results that were positive for a medulloepithelioma. The child was then referred to our institution. Ophthalmic examination revealed vision of hand movements for the right eye, and neovascular glaucoma (intraocular pressure, 35 mm Hg). A sectoral iridectomy was visible inferotemporally, andremnants of the initial tumor were also visible, as a white vascularized tissue invading the angle from the 5-o'clock to 11-o'clock positions. A vitreous hemorrhage prevented perfect visualization of the fundus, which appeared to be normal. The left eye had a visual acuity of 20/30 and showed evidence of a 4-mm-thick ciliary body tumor at the 3-o'clock position, with iris displacement and lens subluxation (Figure 1). An intravitreal cystic mass was visible behind the ciliary body mass; the fundus was otherwise normal. B-scan confirmed the cystic nature of the vitreous mass (Figure 2).

Figure 1.
Ciliary body tumor of the left
eye.

Ciliary body tumor of the left eye.

Figure 2.
B-scan appearance of a cystic
vitreous mass of the left eye.

B-scan appearance of a cystic vitreous mass of the left eye.

The clinical features of both tumors on initial visit were highly suggestive of medulloepithelioma. The left eye tumor was treated with a 125iodine plaque (52 Gy at the apex), with an initial good response. Relapse occurred 1 year later at the margin of the previously treated area and a second 125iodine plaque was used (56.8 Gy at the apex ).

The lesion regressed, and the scar has remained inactive for 6 years, with the patient having a visual acuity of 20/20 OU after surgery on a radiation-induced cataract.

The right eye was finally enucleated after several attempts to treat the residual lesion and neovascular glaucoma. Histological analysis of the right eye confirmed the previous diagnosis of a teratoid malignant medulloepithelioma of the ciliary body with scleral invasion. (Figure 3 and Figure 4).

Figure 3.
A, Features of transcleral extraocular
extension (hematoxylin-eosin–safran, original magnification ×25).
B, Heteroplastic neural tissue with ganglion-like cells (hematoxylin-eosin-safran,
original magnification ×400).

A, Features of transcleral extraocular extension (hematoxylin-eosin–safran, original magnification ×25). B, Heteroplastic neural tissue with ganglion-like cells (hematoxylin-eosin-safran, original magnification ×400).

Figure 4.
A, Areas resembling retinoblastoma
with rosette-like structures associated with epithelial cords and small cysts
(hematoxylin-eosin–safran, original magnification ×200). B, Anastomosing
cords and sheets of nonpigmented epithelial cells (hematoxylin-eosin–safran,
original magnification ×200).

A, Areas resembling retinoblastoma with rosette-like structures associated with epithelial cords and small cysts (hematoxylin-eosin–safran, original magnification ×200). B, Anastomosing cords and sheets of nonpigmented epithelial cells (hematoxylin-eosin–safran, original magnification ×200).

No orbital recurrence has been observed to date (3 years postoperatively).

Comment

Medulloepithelioma is a rare tumor that often leads to enucleation because of its usually large size at diagnosis. We describe an exceptional case of bilateral tumor, which was histologically confirmed as being medulloepithelioma in the right eye. The clinical features of the mass in the left eye on initial visit and the clinical course were highly suggestive of this diagnosis. A biopsy on the left eye was not performed because of the possible risks in a monocular patient. Treatment modalities are difficult to discuss because of the rarity of this entity. Enucleation seems to be the only solution for larger tumors. Small tumors have been successfully treated by surgical excision or Iodine plaques.2,3 The treatment chosen for this child consisted of brachytherapy in the left eye with a 125iodine plaque to avoid intraocular surgery for this monocular child, and a relapse was treated with a second plaque. Unfortunately, the right eye required enucleation. Despite an early relapse near the old scar, treatment has been definitively effective to date, with no evidence of tumor recurrence either in the right orbit or intraocularly in the left eye.

None of the authors have any financial interests in the materials discussed in the article.

Corresponding author and reprints: Laurence Desjardins, MD, Institut CURIE, 26 Rue d'Ulm, 75248 Paris CEDEX 05, France.

References
1.
Zimmerman  LE Verhoeff's "teratoneuroma": a critical reappraisal in light of new observations and current concepts of embryonic tumors. The fourth Frederick H. Verhoeff Lecture. Am J Ophthalmol. 1971;721039- 1057
2.
Broughton  LWZimmerman  LE A clinicopathologic study of intraocular medulloepitheliomas. Am J Ophthalmol. 1978;85407- 418
3.
Shields  JAEagle  RCShields  CLDePotter  P Congenital neoplasms of the nonpigmented ciliary epithelium (medulloepithelioma). Ophthalmology. 1996;1031998- 2006Article
×