Phakomatous choristoma of the eyelid

We report a case of a phakomatous choristoma of the lower eyelid. A subcutaneous tumor was excised from the eyelid. 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 de...

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Bibliographic Details
Published in:Graefe's archive for clinical and experimental ophthalmology 2004-01, Vol.242 (1), p.40-43
Main Authors: Dithmar, Stefan, Schmack, Ingo, Völcker, Hans E, Grossniklaus, Hans E
Format: Article
Language:English
Subjects:
Biomarkers
Child
Choristoma - metabolism
Choristoma - pathology
Choristoma - surgery
Diagnosis, Differential
Eyelid Diseases - metabolism
Eyelid Diseases - pathology
Eyelid Diseases - surgery
Female
Humans
Immunoenzyme Techniques
Lens, Crystalline
S100 Proteins - metabolism
Vimentin - metabolism
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Summary:We report a case of a phakomatous choristoma of the lower eyelid. A subcutaneous tumor was excised from the eyelid. 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. 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.
ISSN:0721-832X
1435-702X
DOI:10.1007/s00417-003-0743-4