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Imaging features of medulloepithelioma: report of four cases and review of the literature

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Abstract

Background

Intraocular medulloepithelioma is a childhood tumor arising from the nonpigmented primitive ciliary neuroepithelium. Although rarer than retinoblastoma, it remains the second most common primary intraocular neoplasm in children. The rarity of intraocular medulloepithelioma creates the challenge in establishing a clinical diagnosis, and radiologically the tumor is often confused with other intraocular masses.

Objective

To describe the clinical, imaging and pathological features of intraocular medulloepithelioma with emphasis on the role of imaging to enable its differentiation from more common intraocular pathology.

Materials and methods

We retrospectively analyzed the clinical, histopathological and imaging data of four children with intraocular medulloepithelioma.

Results

All four children had medulloepithelioma arising from the ciliary body. The children were imaged with US (n = 3), MRI (n = 4), whole-body 99mTc-MDP scintigraphy (n = 2) and CT (n = 1). All four children had enucleation of the involved eye. One tumor was a malignant teratoid variant, two tumors were malignant nonteratoid variants and one was a nonteratoid variant of uncertain malignant potential. None of the tumors had extraocular extension on histopathology or imaging. Two children had associated retinal detachment on US and MRI examinations. All tumors were iso/hyperintense to vitreous on T1-weighted and hypointense on T2-weighted MRI and showed marked contrast enhancement of the solid components. No calcifications were identified on US or CT examinations.

Conclusion

Our findings are consistent with previously reported cases of medulloepithelioma. This series emphasizes the roles of various imaging modalities, with pathological correlation, in differentiating the tumor from other ciliary body masses, in detecting tumor extension and in identifying associated ocular complications. In this series we also describe the results of postsurgical follow-up for tumor recurrence.

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References

  1. Broughton WL, Zimmerman LE (1978) A clinicopathologic study of 56 cases of intraocular medulloepitheliomas. Am J Ophthalmol 85:407–418

    PubMed  CAS  Google Scholar 

  2. Andersen SR (1962) Medulloepithelioma of the retina. Int Ophthalmol Clin 2:483–506

    Article  Google Scholar 

  3. Green WR, Iliff WJ, Trotter RR (1974) Malignant teratoid medulloepithelioma of the optic nerve. Arch Ophthalmol 91:451–454

    Article  PubMed  CAS  Google Scholar 

  4. Brennan RC, Wilson MW, Kaste S et al (2012) US and MRI of pediatric ocular masses with histopathological correlation. Pediatr Radiol 42:738–749

    Article  PubMed  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  6. Zimmerman LE, Font RL, Andersen SR (1972) Rhabdomyosarcomatous differentiation in malignant intraocular medulloepitheliomas. Cancer 30:817–835

    Article  PubMed  CAS  Google Scholar 

  7. Cerase A, De Francesco S, Citterio A et al (2010) Growth of congenital malignant teratoid medulloepithelioma of the ciliary body: a case study. J Neurooncol 96:443–448

    Article  PubMed  Google Scholar 

  8. Saunders T, Margo CE (2012) Intraocular medulloepithelioma. Arch Pathol Lab Med 136:212–216

    Article  PubMed  CAS  Google Scholar 

  9. Priest JR, Williams GM, Manera R et al (2011) Ciliary body medulloepithelioma: four cases associated with pleuropulmonary blastoma – a report from the International Pleuropulmonary Blastoma Registry. Br J Ophthalmol 95:1001–1005

    Article  PubMed  Google Scholar 

  10. Steinkuller PG, Font RL (1997) Congenital malignant teratoid neoplasm of the eye and orbit: a case report and review of the literature. Ophthalmology 104:38–42

    Article  PubMed  CAS  Google Scholar 

  11. Mamalis N, Font RL, Anderson CW et al (1992) Concurrent benign teratoid medulloepithelioma and pineoblastoma. Ophthalmic Surg 23:403–408

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  13. Chung EM, Smirniotopoulos JG, Specht CS et al (2007) From the archives of the AFIP: Pediatric orbit tumors and tumorlike lesions: nonosseous lesions of the extraocular orbit. Radiographics 27:1777–1799

    Article  PubMed  Google Scholar 

  14. Potter PD, Shields CL, Shields JA, Flanders AE (1996) The role of magnetic resonance imaging in children with intraoccular tumors and simulating lesions. Ophthalmology 103:1774–1783

    Google Scholar 

  15. Kaufman LM, Mafee MF, Song CD (1998) Retinoblastoma and simulating lesions. Role of CT, MR imaging and use of Gd-DTPA contrast enhancement. Radiol Clin North Am 36:1101–1117

    Google Scholar 

  16. Foster RE, Murray TG, Byrne SF et al (2000) Echographic features of medulloepithelioma. Am J Ophthalmol 130:364–366

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  18. Galluzzi P, Cerase A, Hadjistilianou T et al (2003) Retinoblastoma: abnormal gadolinium enhancement of anterior segment of eyes at MR imaging with clinical and histopathologic correlation. Radiology 228:683–690

    Article  PubMed  Google Scholar 

  19. Mafee MF, Mafee RF, Malik M et al (2003) Medical imaging in pediatric ophthalmology. Pediatr Clin N Am 50:259–286

    Article  Google Scholar 

  20. Sheth S, Branstetter BF, Escott EJ (2009) Appearance of normal cranial nerves on steady-state free precession MR images. Radiographics 29:1045–1055

    Article  PubMed  Google Scholar 

  21. Seitz J, Held P, Strotzer M et al (2002) Magnetic resonance imaging in patients diagnosed with papilledema: a comparison of 6 different high-resolution T1- and T2(*)-weighted 3-dimensional and 2-dimensional sequences. J Neuroimaging 12:164–171

    Article  PubMed  Google Scholar 

  22. Barr CC, McLean IW, Zimmerman LE (1981) Uveal melanoma in children and adolescents. Arch Ophthalmol 99: 2133–2136.

    Google Scholar 

  23. Peyman GA, Mafee MF (1987) Uveal melanoma and similar lesions: the role of magnetic resonance imaging and computed tomography. Radiol Clin N Am 25:471–486

    PubMed  CAS  Google Scholar 

  24. Chua J, Muen WJ, Reddy A, Brookes J (2012) The masquerades of a childhood ciliary body medulloepithelioma: a case of chronic uveitis, cataract, and secondary glaucoma. Case Rep Ophthalmol Med 2012:493493

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Acknowledgments

This work was supported in part by the American, Lebanese and Syrian Associated Charities and St. Jude Children’s Research Hospital, Memphis, TN, and the Research to Prevent Blindness Inc., New York, NY.

Conflicts of interest

None.

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Authors and Affiliations

  1. Department of Radiological Sciences, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, MS 220, Memphis, TN, 38105-3678, USA

    Rakhee K. Sansgiri, Matthew Wilson, M. Beth McCarville & Kathleen J. Helton

  2. Department of Diagnostic Radiology, Le Bonheur Children’s Hospital, Memphis, TN, USA

    Rakhee K. Sansgiri

  3. Departments of Surgery and Pathology, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Matthew Wilson

  4. Department of Ophthalmology, University of Tennessee Health Science Center, College of Medicine, Memphis, TN, USA

    Matthew Wilson

  5. Department of Radiology, University of Tennessee Health Science Center, College of Medicine, Memphis, TN, USA

    M. Beth McCarville

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  1. Rakhee K. Sansgiri
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  2. Matthew Wilson
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  3. M. Beth McCarville
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  4. Kathleen J. Helton
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Correspondence to Kathleen J. Helton.

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Sansgiri, R.K., Wilson, M., McCarville, M.B. et al. Imaging features of medulloepithelioma: report of four cases and review of the literature. Pediatr Radiol 43, 1344–1356 (2013). https://doi.org/10.1007/s00247-013-2718-x

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  • DOI: https://doi.org/10.1007/s00247-013-2718-x

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