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Updated: Jun 29, 2026
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Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
Primary orbital endodermal sinus tumor (yolk sac tumor)
Hayyam Kiratli1, Kadriye Erkan Balci, Gülnur Güler
1Department of Ophthalmology, Ocular Oncology Service, Hacettepe University School of Medicine, Ankara, Turkey. hkiratli@hacettepe.edu.tr
Summary
Endodermal sinus tumor, also known as yolk sac tumor, is a rare germ cell cancer. This case highlights successful treatment of a primary orbital yolk sac tumor in a young girl.
Area of Science:
- Oncology
- Ophthalmology
- Pediatric Oncology
Background:
- Endodermal sinus tumor (yolk sac tumor) is the most common malignant germ cell neoplasm.
- Typically found in infant testes or ovaries, it can rarely occur in extragonadal sites.
Observation:
- This report details a rare case of primary orbital endodermal sinus tumor in a young female patient.
- The tumor's unusual location in the orbit presented a unique clinical challenge.
Findings:
- The patient underwent surgical exenteration followed by chemotherapy.
- A 9-year disease-free survival was achieved, indicating the effectiveness of the treatment regimen.
Implications:
- This case demonstrates that orbital endodermal sinus tumors, though rare, can be successfully treated.
- Aggressive management involving surgery and chemotherapy offers a favorable prognosis for this rare pediatric malignancy.
