Primary embryonal carcinoma of the orbit in a 10-month-old female: A seven-year follow-up

Purpose: Extra-gonadal germ cell tumors (GCTs) are rare and can be highly aggressive. If correctly identified and treated with multimodality chemotherapy, their prognosis can be significantly improved. We examined a 10 month-old female with primary embryonal carcinoma of the orbit. Design: Case report and literature review. Methods: Case study with 7-year follow-up and literature review of intracranial and intraorbital GCT cases. Results: The patient presented with progressive proptosis and ophthalmoplegia. CT scan revealed an orbital apex mass and biopsy demonstrated a nongerminomatous GCT - an embryonal carcinoma. The patient is tumor-free 7 years after multimodality chemotherapy. She has mild amblyopia and a right micro esotropia. Conclusions: Nongonadal GCTs of the orbit can occur and should be considered in the differential diagnosis of a young child with proptosis and ophthalmoplegia. Five-year survival rates improve significantly with accurate identification and treatment.