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Show Journal of Clinical Neuro- ophthalmology 10( 4): 291- 292, 1990. Feature Photo < 9 1990 Raven Press, Ltd., New York Malignant Teratoid Medulloepithelioma of the Ciliary Body Hugh L. Hennis, M. D., Richard A. Saunders, M. D., and Jerry A. Shields, M. D. A mass arising inferotemporally in the ciliary body of a young boy's eye was diagnosed as medulloepithelioma and followed for 15 months, with documentation by slit lamp photography. At that time growth was noted and the tumor was removed. Histopathologic diagnosis was malignant teratoid medulloepithelioma. Six months afterward there was no evidence of tumor recurrence. Key Words: Eye mass- Medulloepithelioma- Ciliary body- Iridocyclectomy. FIG. 1. Slit lamp photograph of medulloepithelioma of the ciliary body. A lightly pigmented mass, visible ( arrow) behind the iris, displaces the lens nasally. From the Department of Ophthalmology ( H. L. H., R. A. S.), Medical University of South Carolina, Charleston, South Carolina, and the Oncology Service a · A. S.), Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. Address correspondence and reprint requests to Dr. Richard A. Saunders, Storm Eye Institute, 171 Ashley Avenue, Charleston, S. c. 29425- 2236, U. S. A. 291 CASE REPORT A 5- year- old boy was referred because of a mass in his right eye. His visual acuity was 20/ 25 in the right eye and 20/ 15 in the left eye. Slit lamp examination revealed a fleshy, vascular cystic mass arising inferotemporally in the ciliary body. It measured approximately 5 mm in diameter and 3 mm in height, displacing the iris anteriorly and the lens nasally. A clinical diagnosis of medulloepithelioma was made and the patient followed with ocular examinations every 4 months. Fifteen months later tumor growth was noted along with mild opacification of the adjacent posterior lens capsule ( Figs. 1,2). Because of documented progression, the mass was removed by an iridocyclectomy ( Fig. 3). The tumor was composed of anaplastic epithelial cells ( Fig. 4) with foci of brain- like tissue. The histopathologic diagnosis was malignant teratoid medulloepithelioma. One month postoperatively, the patient had best corrected visual acuity of 20170 in the right eye. Six months postoperatively, the posterior subcapsular cataract had progressed, but there was no evidence of tumor recurrence. COMMENT Medulloepithelioma is an ocular tumor derived from the nonpigmented epithelium of the ciliary body ( 1). It is classified teratoid in the presence of heteroplastic elements such as cartilage, skeletal muscle, or brain ( 2). The tumor can be cytolOgically malignant, but distant metastases are rare ( 1- 5). Although enucleation is usually necessary, smaller lesions can be treated by iridocyclectomy or simply 292 H. L. HENNIS ET AL. FIG. 2. B- scan echogram reveals an acoustically solid mass nasally between the iris and lens ( arrow). observed. When managed nonoperatively, serial ocular examinations with photographic documentation are mandatory. In our case, we documented slow growth using slit lamp photography. When FIG. 3. Gross specimen removed at the time of the iridocyclectomy. The segment of uveal tissue measures approximately 6 x 10 mm. Centrally, a whitishtan dome- shaped tumor measuring 4 x 6 x 8 mm is present. growth occurs, surgical excision or enucleation are frequently necessary. REFERENCES 1. Shields JA. Tumors of the nonpigmented ciliary epithelium. In: Diagnosis and management of intraocular tumors. 1st ed. St. Louis: V. Mosby, 1983; 329- 54. 2. Broughton WL, Zimmerman LE. A clinicopathologic study of 56 cases of intraocular medullopeithelioma. Am J OphthalmoI1978; 85: 407- 18. 3. Apple OJ. Rabb MF. Uvea. In: Klein EA, ed. Ocular pathology: clinical applications and self- assessment. St. Louis: CV Mosby, 1985: 229- 32. 4. Brownstein S, Barsoum- Homsy M, Conway VH, et al. Nonteratoid medulloepithelioma of the ciliary body. Ophthalmology 1984; 91: 111~ 22. 5. Shields JA, Shields CL, Schwartz RL. Malignant teratoid medulloepithelioma of the ciliary body simulating persistent hyperplastic primary vitreous. Am J Ophthalmol 1989; 107: 296- 8. FIG. 4. Photomicrograph of malignant teratoid medulloepithelioma of the ciliary body. Centrally, the tumor consists of many poorly differentiated neuroblastic cells with scanty cytoplasm and prominent basophilic nuclei. This central area resembles retinoblastoma with occasional Flexner- Wi ntersteiner- like rosettes ( arrow). ( H& E x250) |
