Intraocular Medulloepitheliomae (Clinical Case Series)

Purpose. Retrospective analysis of clinical, instrumental, cytological and pathological features of intraocular medulloepithelioma based on our own experience in the period from 2005 to 2018.

Patients and Methods. For a specified period of time in the department of ocular oncology and radiology in Moscow Helmholtz Research Institute of Eye Diseases we examined 9 patients aged from 1 to 44 years (5 men and 4 women), with a confirmed histological diagnosis of intraocular medulloepithelioma (medulloblastoma).  The average age of patients in assessing vital status was 15.8 ± 13.5 years, the average age at the time of  making the diagnosis “intraocular neoplasm” was 9.5 ± 11 years. All patients underwent a comprehensive clinical and instrumental ophthalmologic examination, which included visometry, tonometry, perimetry, biomicroscopy, ophthalmoscopy, Doppler ultrasound (ACUSON, USA), as well as general physical examination. Analysis of the initial status (before hospitalization) was carried out retrospectively according to the data of outpatient records. In one case, iridocyclosclerectomy was performed, in eight cases — enucleation of the affected eye followed by histopathological examination, as well as analysis of long-term results of treatment.

Results. A detailed analysis of nine clinical cases is presented, including typical clinical features, echographic signs of a meduloepithelioma (heterogeneous structure of the tumor, anechoic inclusions (cysts) in the  thickness of the tumor) are presented. According to the localization, we observed the tumor of the ciliary body (n = 3), the ciliary body and iris (n = 2), the retina (n = 1), the retina, the choroid and the optic nerve disc (n = 1), the ciliary body and the choroid (n = 1) and the choroid with invasion in extraocular muscles and optic nerve (n = 1). In 8 cases, enucleation was performed, one case was preceded by an iridectomy and a fine- needle aspiration biopsy. One patient underwent local conservative treatment (iridocyclosclerectomy) in the  presence of a tumor of the ciliary body and iris. Pathological and cytological features of the tumor were presented. 8 of 9 patients were alive and had no signs of metastases. One patient died 3 years after treatment.  

Conclusions. Despite the relative rarity, medulloepithelioma should be included in the differential-diagnostic series in patients with suspected retinoblastoma, non-pigmented uveal melanoma, adenocarcinoma of the  retinal pigment epithelium, and other rare intraocular neoplasms. 

1. Zimmerman L.E. Verhoeff ’s “terato‑neuroma”. 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. DOI: 10.1016/0002‑9394(71)91210‑4

2. Fuchs E. Growths and tumors of the ciliary epithelium [in German]. Albrecht Von Graefes Arch Ophthalmol. 1908;68:534–587.

3. Verhoeff F.H. A rare tumor arising from the pars ciliaris retinae (terato‑neuroma) of a nature hitherto unrecognized, and its relation to the so‑called glioma‑retinae. Trans Am Ophthalmol Soc. 1904;10:351–377.

4. Vadmal M., Kahn E., Finger P., Teichberg S. Nonteratoid medulloepithelioma of the retina with electron microscopic and immunohistochemical characterization. Pediatr Pathol Lab Med. 1996;16:663–672. DOI: 10.1080/107710496175598

5. Takei H., Florez L., Moroz K., Bhattacharjee M.B. Medulloepithelioma: two unusual locations. Pathol Int. 2007;57:91–95. DOI: 10.1111/j.1440‑1827.2006.02062.x

6. Morris A.T., Garner A. Medulloepithelioma involving the iris. Br J Ophthalmol. 1975;59:276–278. DOI: 10.1136/bjo.59.5.276

7. Broughton W.L., Zimmerman L.E. A clinicopathologic study of 56 cases of intraocular medulloepitheliomas. Am J Ophthalmol. 1978;85:407–418.

8. Shields J.A., Eagle R.C. Jr., Shields C.L., Potter P.D. Congenital neoplasms of the nonpigmented epithelium (medulloepithelioma). Ophthalmology. 1996;103:1998–2006. DOI: 10.1016/s0161‑6420(96)30394‑1

9. Canning C.R., McCartney A.C., Hungerford J. Medulloepithelioma (diktyoma). Br J Ophthalmol. 1988;72:764–767. DOI: 10.1136/bjo.72.10.764

10. Priest J.R., Williams G.M., Manera R., Jenkinson H., Bründler M.A., Davis S., Murray T.G., Galliani C.A., Dehner L.P. Ciliary body medulloepithelioma: four cases associated with pleuropulmonary blastomae a report from the International Pleuropulmonary Blastoma Registry. Br J Ophthalmol. 2011;95:1001–1005. DOI: 10.1136/bjo.2010.189779

11. Badel A.J., Lagrange F. Carcinome primitif de procès et du corps ciliare. Arch Ophthalmol (Paris). 1892;12:143–148.

12. Grinker R.R. Gliomas of the retina including results of studies with silver impregnations. Arch Ophthalmol. 1931;5:920–935. DOI: 10.1001/archopht.1931.00820060088009

13. Saakyan S.V., Tsygankov A.Yu., Ivanova O.A., Zaharova G.P., Isaeva R.T., Moiseeva N.I., Karamysheva A.F. Malignant medulloepithelioma mimicking retinoblastoma — clinical and morphological case analysis and cell culture experience. The Russian Annals of Ophthalmology = Vestnik oftal’mologii. 2016;132(6):64–69 (In Russ.). DOI: 10.17116/oftalma2016132664‑69

14. Carrillo R., Streeten B.W. Malignant teratoid medulloepithelioma in an adult. Arch Ophthalmol. 1979;97:695–699. DOI: 10.1001/archopht.1979.01020010347012

15. Jumper M.J., Char D.H., Bitner D.G. Neglected malignant medulloepithelioma of the eye. Orbit. 1999;18:37–43. DOI: 10.1076/orbi.18.1.37.2726

16. Litricin O., Latkovic Z. Malignant teratoid medulloepithelioma in an adult. Ophthalmologica. 1985;191:17–21. DOI: 10.1159/000309533

17. Zimmerman L.E., Font R.L., Andersen S.R. Rhabdomyosarcomatous differentiation in malignant intraocular medulloepitheliomas. Cancer. 1972;30:817–835. DOI: 10.1002/1097‑0142(197209)30:3<817::aid‑cncr2820300334>3.0.co;2‑e

18. Ali M.J., Honavar S.G., Vemuganti G.K. Ciliary body medulloepithelioma in an adult. Surv Ophthalmol. 2013;58(3):266–272. DOI: 10.1016/j.survophthal.2012.08.006

19. Mahdjoubi A., Cassoux N., Levy‑Gabriel C., Desjardins L., Klos J., Caly M., Vlajnic T., Gardrat S., Klijanienko J. Adult ocular medulloepithelioma diagnosed by transscleral fine needle aspiration: A case report. Diagn Cytopathol. 2017;45(6):561–564. DOI: 10.1002/dc.23694

20. Sosińska‑Mielcarek K., Senkus‑Konefka E., Jaskiewicz K., Kordek R., Jassem J. Intraocular malignant teratoid medulloepithelioma in an adult: clinicopathological case report and review of the literature. Acta Ophthalmol Scand. 2006;84(2):259–262. DOI: 10.1111/j.1600‑0420.2005.00586.x

21. Horoshilova‑Maslova I.P., Saakyan S.V., Nabieva M.K., Zaharova G.P., Amiryan A.G. Adenocarcinoma of retinal pigment epithelium (a clinical and histopathological case report). Russian ophthalmological journal = Rossijskiy oftal’mologicheskiy zhurnal. 2011;4(2):64–68 (In Russ.).

22. Alkatan H., Al‑Amry M., Al‑Hussain H., Al‑Dhibi H., Al‑Mesfer S. Medulloepithelioma of the ciliary body: the delay in diagnosis and frequent initial mismanagement. Can J Ophthalmol. 2011;46:431–438. DOI: 10.1016/j.jcjo.2011.07.007

23. Gündüz K., Hosal B.M., Zilelioglu G., Günalp I. The use of ultrasound biomicroscopy in the evaluation of anterior segment tumors and simulating conditions. Ophthalmologica. 2007;221:305– 312. DOI: 10.1159/000104760

24. Font R.L., Rishi K. Diffuse retinal involvement in malignant nonteratoid medulloepithelioma of ciliary body in an adult. Arch Ophthalmol. 2005;123:1136–1138. doi: 10.1001/archopht.123.8.1136

25. Davidorf F.H., Craig E., Birnbaum L., Wakely P. Jr. Management of medulloepithelioma of the ciliary body with brachytherapy. Am J Ophthalmol. 2002;133:841–843.

26. Ramasubramanian A., Shields C.L., Kytasty C., Mahmood Z., Shah S.U., Shields J.A. Resection of intraocular tumors (partial lamellar sclerouvectomy) in the pediatric age group. Ophthalmology. 2012;119:2507–2513. DOI: 10.1016/j.ophtha.2012.06.044

27. Meel R., Chawla B., Mohanti B.K., Kashyap S., Bakhshi S. Ocular medulloepithelioma chemosensitivity [letter]. Ophthalmology. 2010;117:2440. DOI: 10.1016/j.ophtha.2010.05.008