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Presumed Bilateral Medulloepithelioma
Arch Ophthalmol. 2001;119:449-450.
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.1-3
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).
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Figure 1. Ciliary body tumor of the left
eye.
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Figure 2. B-scan appearance of a cystic
vitreous mass of the left eye.
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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).
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Figure 3. A, Features of transcleral extraocular
extension (hematoxylin-eosin–safran, original magnification x25).
B, Heteroplastic neural tissue with ganglion-like cells (hematoxylin-eosin-safran,
original magnification x400).
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Figure 4. A, Areas resembling retinoblastoma
with rosette-like structures associated with epithelial cords and small cysts
(hematoxylin-eosin–safran, original magnification x200). B, Anastomosing
cords and sheets of nonpigmented epithelial cells (hematoxylin-eosin–safran,
original magnification x200).
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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.
AUTHOR INFORMATION
None of the authors have any financial interests in the materials discussed
in the article.
Livia Lumbroso, MD;
Laurence Desjardins, MD;
Olivier Coue, MD
Paris, France
Yvette Ducourneau, MD;
Alain Pechereau, MD, PhD
Nantes, France
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;72:1039-1057.
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2. Broughton LW, Zimmerman LE. A clinicopathologic study of intraocular medulloepitheliomas. Am J Ophthalmol. 1978;85:407-418.
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3. Shields JA, Eagle RC, Shields CL, DePotter P. Congenital neoplasms of the nonpigmented ciliary epithelium (medulloepithelioma). Ophthalmology. 1996;103:1998-2006.
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