Skip to main content

Intraocular Medulloepithelioma

  • Chapter
  • First Online:
Ocular Oncology

Part of the book series: Retina Atlas ((RA))

  • 614 Accesses

Abstract

Intraocular medulloepithelioma is a rare embryonal neuroepithelial tumor that originates from the undifferentiated medullary epithelium over the ciliary body or inner layer of the optic cup (Zimmerman 1971; Fuchs 1908). It commonly arises from the nonpigmented ciliary epithelium of the pars plicata and rarely in the iris, retina, optic nerve, and the central nervous system (Verhoeff 1904; Takei et al. 2007; Morris and Garner 1975; Vadmal et al. 1996; Green et al. 1974; Chavez et al. 2004; Sundaram et al. 2003; Steinkuller and Font 1997).

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • 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;46:431–8.

    Article  Google Scholar 

  • Ayres B, Brasil OM, Klejnberg C, et al. Ciliary body medulloepithelioma: clinical, ultrasound biomicroscopic and histopathologic correlation. Clin Exp Ophthalmol. 2006;34:695–8.

    Article  Google Scholar 

  • Barr CC, McLean IW, Zimmerman LE. Uveal melanoma in children and adolescents. Arch Ophthalmol. 1981;99:2133–6.

    Article  CAS  Google Scholar 

  • Broughton WL, Zimmerman LE. A clinicopathologic study of 56 cases of intraocular medulloepitheliomas. Am J Ophthalmol. 1978;85:407–18.

    Article  CAS  Google Scholar 

  • Canning CR, McCartney AC, Hungerford J. Medulloepithelioma (diktyoma). Br J Ophthalmol. 1988;72:764–7.

    Article  CAS  Google Scholar 

  • Cassoux N, Charlotte F, Sastre X, et al. Conservative surgical treatment of medulloepithelioma of the ciliary body [letter]. Arch Ophthalmol. 2010;128:380–1.

    Article  Google Scholar 

  • Chavez M, Mafee MF, Castillo B, et al. Medulloepithelioma of the optic nerve. J Pediatr Ophthalmol Strabismus. 2004;41:48–52.

    Article  Google Scholar 

  • Davidorf FH, Craig E, Birnbaum L, Wakely P Jr. Management of medulloepithelioma of the ciliary body with brachytherapy. Am J Ophthalmol. 2002;133:841–3.

    Article  Google Scholar 

  • Floyd BB, Minckler DS, Valentin L. Intraocular medulloepithelioma in a 79-year-old man. Ophthalmology. 1982;89:1088–94.

    Article  CAS  Google Scholar 

  • Font RL, Rishi K. Diffuse retinal involvement in malignant nonteratoid medulloepithelioma of ciliary body in an adult. Arch Ophthalmol. 2005;123:1136–8.

    Article  Google Scholar 

  • Fuchs E. Growths and tumors of the ciliary epithelium [in German]. Albrecht Von Graefes Arch Ophthalmol. 1908;68:534–87.

    Article  Google Scholar 

  • Gopal L, Babu K, Gupta S, et al. Pigmented malignant medulloepithelioma of the ciliary body. J Pediatr Ophthalmol Strabismus. 2004;41:364–6.

    Article  Google Scholar 

  • Green WR, Iliff WJ, Trotter RR. Malignant teratoid medulloepithelioma of the optic nerve. Arch Ophthalmol. 1974;91:451–4.

    Article  CAS  Google Scholar 

  • Grinker RR. Gliomas of the retina, including the results of studies with silver impregnations. Arch Ophthalmol. 1931;5:930–5.

    Article  Google Scholar 

  • Gündüz K, Hoşal BM, Zilelioğlu G, et al. The use of ultrasound biomicroscopy in the evaluation of anterior segment tumors and simulating conditions. Ophthalmologica. 2007;221:305–12.

    Article  Google Scholar 

  • Gupta NK, Simon JW, Walton DS, et al. Bilateral ectopia lentis as a presenting feature of medulloepithelioma. J AAPOS. 2001;5:255–7.

    Article  CAS  Google Scholar 

  • Husain SE, Husain N, Boniuk M, et al. Malignant nonteratoid medulloepithelioma of the ciliary body in an adult. Ophthalmology. 1998;105:596–9.

    Article  CAS  Google Scholar 

  • Jumper MJ, Char DH, Howes EL Jr, et al. Neglected malignant medulloepithelioma of the eye. Orbit. 1999;18:37–43.

    Article  Google Scholar 

  • Kaliki S, Eagle RC, Grossniklaus HE, et al. Inadvertent implantation of aqueous tube shunts in glaucomatous eyes with unrecognized intraocular neoplasms: a report of 5 cases. JAMA Ophthalmol. 2013a;131:925–8.

    Article  Google Scholar 

  • Kaliki S, Shields CL, Eagle RC Jr, et al. Ciliary body medulloepithelioma: analysis of 41 cases. Ophthalmology. 2013b;120:2552–9.

    Article  Google Scholar 

  • Kanavi MR, Soheilian M, Kamrava K, et al. Medulloepithelioma masquerading as chronic anterior granulomatous uveitis. Can J Ophthalmol. 2007;42:474–6.

    Article  Google Scholar 

  • Litricin O, Latkovic Z. Malignant teratoid medulloepithelioma in an adult. Ophthalmologica. 1985;191:17–21.

    Article  CAS  Google Scholar 

  • Lumbroso L, Disjardins L, Coue O, et al. Presumed bilateral medulloepithelioma. Arch Ophthalmol. 2001;119:449–50.

    CAS  PubMed  Google Scholar 

  • Mamalis N, Font RL, Anderson CW, et al. Concurrent benign teratoid medulloepithelioma and pineoblastoma. Ophthalmic Surg. 1992;23:403–8.

    CAS  PubMed  Google Scholar 

  • Meel R, Chawla B, Mohanti BK, et al. Ocular medulloepithelioma chemosensitivity. Ophthalmology. 2010;117:2440.

    Article  Google Scholar 

  • Minoda K, Hirose Y, Sugano I, et al. Occurrence of sequential intraocular tumors: malignant medulloepithelioma subsequent to retinoblastoma. Jpn J Ophthalmol. 1993;37:293–300.

    CAS  PubMed  Google Scholar 

  • Morris AT, Garner A. Medulloepithelioma involving the iris. Br J Ophthalmol. 1975;59:276–8.

    Article  CAS  Google Scholar 

  • Peyman GA, Mafee MF. Uveal melanoma and similar lesions: the role of magnetic resonance imaging and computed tomography. Radiol Clin N Am. 1987;25:471–86.

    CAS  PubMed  Google Scholar 

  • Poon DS, Reich E, Smith VM, et al. Ruthenium-106 plaque brachytherapy in the primary management of ocular medulloepithelioma. Ophthalmology. 2015;122:1949–51.

    Article  Google Scholar 

  • Potter PD, Shields CL, Shields JA, et al. The role of magnetic resonance imaging in children with intraocular tumors and simulating lesions. Ophthalmology. 1996;103:1774–83.

    Article  CAS  Google Scholar 

  • Priest JR, Williams GM, Manera R, et al. Ciliary body medulloepithelioma: four cases associated with pleuropulmonary blastomaea report from the International Pleuropulmonary Blastoma Registry. Br J Ophthalmol. 2011;95:1001–5.

    Article  Google Scholar 

  • Pushker N, Khuraijam N, Sen S, et al. Medulloepithelioma of the ciliary body associated with massive intravitreal hemorrhage in an adult [letter]. Can J Ophthalmol. 2008;43:253–4.

    Article  Google Scholar 

  • Ramasubramanian A, Shields CL, Kytasty C, et al. Resection of intraocular tumors (partial lamellar sclerouvectomy) in the pediatric age group. Ophthalmology. 2012;119:2507–13.

    Article  Google Scholar 

  • Sansgiri RK, Wilson M, McCarville M, et al. Imaging features of medulloepithelioma: report of four cases and review of the literature. Pediatr Radiol. 2013;43:1344–56.

    Article  Google Scholar 

  • 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;249:1259–61.

    Article  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • Shields JA, Eagle RC Jr, Shields CL, et al. Fluorescein angiography and ultrasonography of malignant intraocular medulloepithelioma. J Pediatr Ophthalmol Strabismus. 1996a;33:193–6.

    CAS  PubMed  Google Scholar 

  • Shields JA, Eagle RC Jr, Shields CL, et al. Congenital neoplasms of the nonpigmented epithelium (medulloepithelioma). Ophthalmology. 1996b;103:1998–2006.

    Article  CAS  Google Scholar 

  • Shields JA, Eagle RC Jr, Shields CL, et al. Pigmented medulloepithelioma of the ciliary body. Arch Ophthalmol. 2002;120:207–10.

    PubMed  Google Scholar 

  • Singh A, Singh AD, Shields CL, et al. Iris neovascularization in children as a manifestation of underlying medulloepithelioma. J Pediatr Ophthalmol Strabismus. 2001;38:224–8.

    CAS  PubMed  Google Scholar 

  • Spicer WT, Greeves RA. Multiple cysts in the anterior chamber derived from a congenital cystic growth of the ciliary epithelium. Proc R Soc Med. 1915;8:9–26.

    CAS  PubMed  PubMed Central  Google Scholar 

  • Steinkuller PG, Font RL. Congenital malignant teratoid neoplasm of the eye and orbit. Ophthalmology. 1997;104:38–42.

    Article  CAS  Google Scholar 

  • Sundaram C, Vydehi V, Jaganmohan Reddy J, et al. Medulloepithelioma: a case report. Neurol India. 2003;51:546–7. Br J Ophthalmol 59:276–8

    CAS  PubMed  Google Scholar 

  • Takei H, Florez L, Moroz K, et al. Medulloepithelioma: two unusual locations. Pathol Int. 2007;57:91–5.

    Article  CAS  Google Scholar 

  • Vadmal M, Kahn E, Finger P, et al. Nonteratoid medulloepithelioma of the retina with electron microscopic and immunohistochemical characterization. Pediatr Pathol Lab Med. 1996;16:663–72.

    Article  CAS  Google Scholar 

  • Vajaranant TS, Mafee MF, Kapur R, et al. Medulloepithelioma of the ciliary body and optic nerve: clinicopathologic, CT and MR imaging features. Neuroimaging Clin N Am. 2005;15:69–83.

    Article  Google Scholar 

  • 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;10:351–77.

    CAS  PubMed  PubMed Central  Google Scholar 

  • Wolter JR, James BR. Adult type of medullo-epithelioma of the ciliary body. Am J Ophthalmol. 1958;46:19–26.

    Article  CAS  Google Scholar 

  • Zhou M, Xu G, Bojanowski CM, et al. Differential diagnosis of anterior chamber cysts with ultrasound biomicroscopy: Ciliary body medulloepithelioma. Acta Ophthalmol Scand. 2006;84:137–9.

    Article  Google Scholar 

  • 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;72:1039–57.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Gupta, S.R., Kaliki, S. (2019). Intraocular Medulloepithelioma. In: Rojanaporn, D. (eds) Ocular Oncology. Retina Atlas. Springer, Singapore. https://doi.org/10.1007/978-981-13-2336-2_15

Download citation

  • DOI: https://doi.org/10.1007/978-981-13-2336-2_15

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-13-2335-5

  • Online ISBN: 978-981-13-2336-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics