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 ...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: NongonadalGCTs 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.展开更多
文摘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: NongonadalGCTs 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.
