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Phakomatous choristoma of the eyelid

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Abstract

Background

We report a case of a phakomatous choristoma of the lower eyelid.

Methods

A subcutaneous tumor was excised from the eyelid.

Results

A 12-month-old girl was referred because of a tumor in her right lower eyelid, which had been present since birth. The subcutaneous tumor had remained unchanged and was considered a dermoid cyst. No other abnormalities were present. The tumor was excised. Histologically, the tumor consisted of abnormal lens tissue. The immunohistochemical profile included strong immunoreactivity for vimentin and S-100.

Conclusion

Phakomatous choristoma is an extraordinary rare tumor that probably develops from an abnormal separation or migration of cells from the lens placode into the mesodermal structures of the lid. Only 18 cases have been described so far, less than 5 in the European literature. This benign tumor may be confused with cutaneous adnexal neoplasms.

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Authors and Affiliations

  1. Department of Ophthalmology, University of Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany

    Stefan Dithmar, Ingo Schmack & Hans E. Völcker

  2. Department of Ophthalmology, Emory University School of Medicine, Atlanta, GA, USA

    Hans E. Grossniklaus

Authors
  1. Stefan Dithmar
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  2. Ingo Schmack
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  3. Hans E. Völcker
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  4. Hans E. Grossniklaus
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Correspondence to Stefan Dithmar.

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Dithmar, S., Schmack, I., Völcker, H.E. et al. Phakomatous choristoma of the eyelid. Graefe's Arch Clin Exp Ophthalmol 242, 40–43 (2004). https://doi.org/10.1007/s00417-003-0743-4

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  • DOI: https://doi.org/10.1007/s00417-003-0743-4

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