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Vol 4 (2019): Continuous Publishing
Review paper
Published online: 2019-12-06
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Characteristics, management, and outcome of ocular medulloepithelioma: systematic review of literature and case report

Yacoub A. Yousef1, Shatha Hajja2, Hanood AbuRass3, Mays AlJboor1, Rashed Mustafa Nazzal4, Katarzyna Nowomiejska5, Hussam Haddad3, Iyad Sultan2
DOI: 10.5603/OJ.2019.0015
·
Ophthalmol J 2019;4:92-99.
Affiliations
  1. Department of Surgery (Ophthalmology), King Hussein Cancer Centre, Amman, Jordan
  2. Department of Paediatric Oncology, King Hussein Cancer Centre, Amman, Jordan
  3. Department of Pathology, King Hussein Cancer Centre, Amman, Jordan
  4. Department of Surgery/Ophthalmology, King Hussein Cancer Center (KHCC), Amman, Jordan
  5. Department of Ophthalmic Surgery, Medical university of Lublin, Lublin, Poland

open access

Vol 4 (2019): Continuous Publishing
REVIEW
Published online: 2019-12-06

Abstract

Background: Ocular medulloepithelioma (OM) is a rare ocular malignancy. This is a report of a rare case of medulloepithelioma that was misdiagnosed as retinoblastoma.

Material and methods: A case report and review of published, peer-reviewed, English language literature reporting on ocular medulloepithelioma.

Results: A seven-year-old girl presented with a white mass in the anterior chamber of her left eye. The initial diagnosis was retinoblastoma with anterior chamber invasion, and therefore it was enucleated. Microscopy showed a cellular tumour composed of malignant primitive cells forming sheets, rosettes, and tubular structures. Based on the presence of prominent pleomorphism the tumour was diagnosed as malignant teratoid medulloepithelioma. At last date of follow-up three years after enucleation, the patient was alive without metastasis. A systematic review of literature, analysed 177 cases of OM. The tumour was localised in the ciliary body in 134 (92%) cases, and 26 (23%) cases had extraocular extension. Primary management was enucleation in 84 (55%) cases, tumour resection in 32 (21%) cases, and radiation therapy in 20 (13%) cases. Histopathology disclosed benign features in 36 (22%) eyes, malignant features in 124 (78%) eyes, teratoid features in 72 (59%), and non-teratoid features in 51 (41%) eyes. At a mean follow-up of five years, 14 (8%) patients had metastasis, and six (6%) patients were dead.

Conclusions: Ocular medulloepithelioma most commonly affects children. The most common type is the malignant
teratoid type, but it has a favourable prognosis, and distant metastasis and mortality are relatively rare, at 8%
and 6%, respectively.

Abstract

Background: Ocular medulloepithelioma (OM) is a rare ocular malignancy. This is a report of a rare case of medulloepithelioma that was misdiagnosed as retinoblastoma.

Material and methods: A case report and review of published, peer-reviewed, English language literature reporting on ocular medulloepithelioma.

Results: A seven-year-old girl presented with a white mass in the anterior chamber of her left eye. The initial diagnosis was retinoblastoma with anterior chamber invasion, and therefore it was enucleated. Microscopy showed a cellular tumour composed of malignant primitive cells forming sheets, rosettes, and tubular structures. Based on the presence of prominent pleomorphism the tumour was diagnosed as malignant teratoid medulloepithelioma. At last date of follow-up three years after enucleation, the patient was alive without metastasis. A systematic review of literature, analysed 177 cases of OM. The tumour was localised in the ciliary body in 134 (92%) cases, and 26 (23%) cases had extraocular extension. Primary management was enucleation in 84 (55%) cases, tumour resection in 32 (21%) cases, and radiation therapy in 20 (13%) cases. Histopathology disclosed benign features in 36 (22%) eyes, malignant features in 124 (78%) eyes, teratoid features in 72 (59%), and non-teratoid features in 51 (41%) eyes. At a mean follow-up of five years, 14 (8%) patients had metastasis, and six (6%) patients were dead.

Conclusions: Ocular medulloepithelioma most commonly affects children. The most common type is the malignant
teratoid type, but it has a favourable prognosis, and distant metastasis and mortality are relatively rare, at 8%
and 6%, respectively.

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Keywords

ciliary body; iris; medulloepithelioma; retinoblastoma; tumour

About this article
Title

Characteristics, management, and outcome of ocular medulloepithelioma: systematic review of literature and case report

Journal

Ophthalmology Journal

Issue

Vol 4 (2019): Continuous Publishing

Article type

Review paper

Pages

92-99

Published online

2019-12-06

Page views

712

Article views/downloads

583

DOI

10.5603/OJ.2019.0015

Bibliographic record

Ophthalmol J 2019;4:92-99.

Keywords

ciliary body
iris
medulloepithelioma
retinoblastoma
tumour

Authors

Yacoub A. Yousef
Shatha Hajja
Hanood AbuRass
Mays AlJboor
Rashed Mustafa Nazzal
Katarzyna Nowomiejska
Hussam Haddad
Iyad Sultan

References (33)
  1. Zimmerman LE. 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(6): 1039–1057.
    PubMed
  2. Zimmerman LE, Broughton WLA. Clinicopathologic and follow up study of fifty-six cases of intraocular medulloepithelioma. In: Jakobiec FA. ed. Ocular Adnexal Tumors. Aesculapius Publishing, Birmingham, AL 1978: 181–196.
  3. Verhoeff FH. 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(Pt 2): 351–377.
    PubMed
  4. Vadmal M, Kahn E, Finger P, et al. Nonteratoid medulloepithelioma of the retina with electron microscopic and immunohistochemical characterization. Pediatr Pathol Lab Med. 1996; 16(4): 663–672.
    PubMed
  5. Takei H, Florez L, Moroz K, et al. Medulloepithelioma: Two unusual locations. Pathol Int. 2007; 57(2): 91–95.
    CrossRefPubMed
  6. Shields J, Eagle R, Shields C, et al. Congenital Neoplasms of the Nonpigmented Ciliary Epithelium (medulloepithelioma). Ophthalmology. 1996; 103(12): 1998–2006.
    CrossRef
  7. Broughton WL, Zimmerman LE. A clinicopathologic study of 56 cases of intraocular medulloepitheliomas. Am J Ophthalmol. 1978; 85(3): 407–418.
    CrossRefPubMed
  8. Shields J, Eagle R, Shields C, et al. Congenital Neoplasms of the Nonpigmented Ciliary Epithelium (medulloepithelioma). Ophthalmology. 1996; 103(12): 1998–2006.
    CrossRef
  9. Shields JA, Shields CL. Tumors of the nonpigmented ciliary epithelium. In: Shields JA, Shields CL. ed. Intraocular Tumors: An Atlas and Textbook. 2nd ed. Lippincott Williams and Wilkins, Philadelphia, PA 2008: 482–490.
  10. Kaliki S, Shields C, Eagle R, et al. Ciliary Body Medulloepithelioma. Ophthalmology. 2013; 120(12): 2552–2559.
    CrossRef
  11. Priest JR, Williams GM, Manera R, et al. Ciliary body medulloepithelioma: four cases associated with pleuropulmonary blastoma — a report from the International Pleuropulmonary Blastoma Registry. Br J Ophthalmol. 2011; 95(7): 1001–1005.
    CrossRefPubMed
  12. Fuchs E. Growths and tumors of the ciliary epithelium [in German]. Albrecht Von Graefes Arch Ophthalmol. 1908; 68: 534–87.
  13. Grinker RR. Gliomas of the retina including results of studies with silver impregnations. Arch Ophthalmol. 1931; 5: 920–935.
    CrossRef
  14. Pendergrass TW, Davis S. Incidence of retinoblastoma in the United States. Arch Ophthalmol. 1980; 98(7): 1204–1210.
    CrossRefPubMed
  15. Litricbin O. Latkovi_c Z. Malignant teratoid medulloepithelioma in an adult. Ophthalmologica. 1985; 191: 17–21.
  16. Floyd BB, Minckler DS, Valentin L. Intraocular medulloepithelioma in a 79-year-old man. Ophthalmology. 1982; 89(9): 1088–1094.
    CrossRefPubMed
  17. Pushker N, Khuraijam N, Sen S, et al. Medulloepithelioma of the ciliary body associated with massive intravitreal hemorrhage in an adult. Can J Ophthalmol. 2008; 43(2): 253–254.
    CrossRefPubMed
  18. Wolter JR, James BR. Adult type of medullo-epithelioma of the ciliary body. Am J Ophthalmol. 1958; 46(1 Pt 1): 19–26.
    CrossRefPubMed
  19. Broughton WL, Zimmerman LE. A clinicopathologic study of 56 cases of intraocular medulloepitheliomas. Am J Ophthalmol. 1978; 85(3): 407–418.
    CrossRefPubMed
  20. Kaliki S, Eagle RC, Grossniklaus HE, et al. Inadvertent implantation of aqueous tube shunts in glaucomatous eyes with unrecognized intraocular neoplasms: report of 5 cases. JAMA Ophthalmol. 2013; 131(7): 925–928.
    CrossRefPubMed
  21. Alkatan H, Al-Amry M, Al-Hussain H, et al. Medulloepithelioma of the ciliary body: the delay in diagnosis and frequent initial mismanagement. Can J Ophthalmol. 2011; 46(5): 431–438.
    CrossRefPubMed
  22. Sharma P, Shields CL, Turaka K, et al. Ciliary body medulloepithelioma with neoplastic cyclitic membrane imaging with fluorescein angiography and ultrasound biomicroscopy. Graefes Arch Clin Exp Ophthalmol. 2011; 249(8): 1259–1261.
    CrossRefPubMed
  23. Shields JA, Eagle RC, Shields CL, et al. Fluorescein angiography and ultrasonography of malignant intraocular medulloepithelioma. J Pediatr Ophthalmol Strabismus. 1996; 33: 193–196.
  24. Shields JA, Shields CL, Schwartz RL. Malignant teratoid medulloepithelioma of the ciliary body simulating persistent hyperplastic primary vitreous. Am J Ophthalmol. 1989; 107(3): 296–298.
    CrossRefPubMed
  25. Font RL, Rishi K. Diffuse retinal involvement in malignant nonteratoid medulloepithelioma of ciliary body in an adult. Arch Ophthalmol. 2005; 123(8): 1136–1138.
    CrossRefPubMed
  26. Carrillo R, Streeten BW. Malignant teratoid medulloepithelioma in an adult. Arch Ophthalmol. 1979; 97(4): 695–699.
    CrossRefPubMed
  27. Kivelä T, Summanen P. Possible shared pathogenesis of the retinoinvasive phenotype of malignant medulloepithelioma and malignant melanoma of the ciliary body. Arch Ophthalmol. 2006; 124(7): 1066; author reply 1067.
    CrossRefPubMed
  28. Shields J, Eagle R, Shields C, et al. Acquired Neoplasms of the Nonpigmented Ciliary Epithelium (adenoma and adenocarcinoma). Ophthalmology. 1996; 103(12): 2007–2016.
    CrossRef
  29. Monchy D, Jacquiot P, Dubourdieu D, et al. [Malignant teratoid medulloepithelioma of the ciliary body. Apropos of a case and review of the literature]. Arch Anat Cytol Pathol. 1993; 41(2): 107–112.
    PubMed
  30. Cassoux N, Charlotte F, Sastre X, et al. Conservative surgical treatment of medulloepithelioma of the ciliary body. Arch Ophthalmol. 2010; 128(3): 380–381.
    CrossRefPubMed
  31. Ramasubramanian A, Shields CL, Kytasty C, et al. Resection of intraocular tumors (partial lamellar sclerouvectomy) in the pediatric age group. Ophthalmology. 2012; 119(12): 2507–2513.
    CrossRefPubMed
  32. Meel R, Chawla B, Mohanti BK, et al. Ocular medulloepithelioma chemosensitivity. Ophthalmology. 2010; 117(12): 2440.e1–2440.e2.
    CrossRefPubMed
  33. Hu HM, Zhang WL, Wang YZ, et al. [Clinical features, treatment and prognosis of retinoblastoma in distant metastasis stage]. Zhonghua Yan Ke Za Zhi. 2017; 53(2): 121–127.
    CrossRefPubMed

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