Germ cell tumor can affect extragonadal sites. Teratoma is a well-recognized extragonadal tumor in the orbit. Primary yolk sac tumor (YST) or endodermal sinus tumor of orbit is rare and only few cases have been reported in the literature. Its clinical presentation may mimic many common pediatric orbital conditions, and delay in diagnosis affects ocular morbidity and mortality. In the past orbital YST has been treated with multimodal therapy including surgery, systemic chemotherapy, and radiotherapy. Herein we describe a case of primary orbital YST and reviewed the literature for similar cases. The review aims to describe the clinical presentation, imaging features, histopathological characteristics, and management of orbital YST.
Keywords: Eye; Germ cell tumor; Orbit; Orbital yolk sac tumor; Tumor; Yolk sac carcinoma.