Isolated orbital endodermal sinus tumor.

PURPOSE: To describe the clinical, serologic, histopathologic, and immunohistochemical findings of an isolated endodermal sinus tumor of the orbit in a 1-year-old boy. METHODS: A retrospective case report and literature review. RESULTS: The patient was managed by the ophthalmology and oncology services. After the diagnosis was established by serologic analysis and histopathology, the patient underwent chemotherapy. CONCLUSIONS: Endodermal sinus tumor is a malignant germ-cell tumor that usually involves the gonadal tissue. Primary extragonadal sites occur rarely, and include the orbit. Treatment options include surgical removal and chemotherapy.