J Korean Ophthalmol Soc.
2007 Apr;48(4):604-610.
Primary Yolk Sac Tumor of the Orbit
- Affiliations
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- 1Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University College of Medicine, Seoul, Korea. ydkim@smc.samsung.co.kr
- 2Department of Ophthalmology, Korea Veterans Hospital, Seoul, Korea.
Abstract
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PURPOSE: To report a case of primary yolk sac tumor of the orbit.
METHODS
A 15-month-old girl was referred for evaluation of ptosis and strabismus in the right eye which had developed over the previous two weeks. On examination, ptosis, exotropia, proptosis, and restriction of extraocular muscle movement of the right eye were noted. A computed tomography (CT) scan of the orbit showed a 2.4 cm x 3.4 cm-sized soft tissue mass in the posterior aspect of the right orbit which enveloped the optic nerve and invaded the maxillary sinus, posterior ethmoid sinus, nasal cavity, and intracranial space. Endoscopic biopsy of the nasal cavity mass was performed.
RESULTS
Histologic evaluation revealed solid proliferation of germ cells with large, clear cytoplasm and intracellular, extracellular hyaline globules. Immunohistochemical staining for alpha-fetoprotein was positive. Results of further systemic evaluation were all negative. The tumor regressed after combined chemotherapy and radiotherapy. The patient has been disease free for 2 years.
CONCLUSIONS
The possibility of yolk sac tumor should be considered in the differential diagnosis in a younger patient for an orbital mass with rapidly increasing size and adjacent bony destruction. Prompt diagnosis and appropriate therapeutic approaches are essential.
