Abstract
To present a unique case of lymphoepithelial carcinoma originating from the orbit, an exceedingly rare manifestation with no precedent in English literature. A 49-year-old male patient exhibited progressive right eye bulging, blurred vision, diplopia, and ophthalmoplegia. Imaging revealed a tumor involving the orbital space, nasolacrimal duct, and maxillary sinus. The patient underwent tumor excision, revealing lymphoepithelial carcinoma on histopathology. Immunohistochemistry supported the diagnosis with EBV reactivity. After then, the patient received concurrent chemoradiotherapy following orbital tumor excision. Subsequent imaging indicated tumor regression over orbit and periocular regions. Although parotid gland metastasis was detected, we arranged parotidectomy and adjuvant therapy, resulting in no recurrence and symptomatic improvement six months after treatment. This case extends the existing understanding of clinical manifestation of lymphoepithelial carcinoma. The unique presentation of lymphoepithelial carcinoma originating from the orbit emphasizes the importance of considering rare manifestations when approaching complex clinical scenarios. The correlation between EBV infection and lymphoepithelial carcinoma in the context of Asian populations was discussed, although not entirely confirmed in this case. Treatment strategies were influenced by the rarity of the tumor, combining surgical resection, induction chemotherapy, and concurrent chemoradiotherapy. This report presents a case of lymphoepithelial carcinoma originating from the orbit, adding to the understanding of the clinical spectrum of this rare malignancy. The management showcases a multidisciplinary strategy that considers the tumor's rarity and aggressive behavior. Given the lack of established guidelines, further research of cases are essential to optimize the treatment of lymphoepithelial carcinoma across diverse presentations.