Nasopharyngeal carcinoma presenting with no light perception vision

We report a case of a 61-year-old female visiting from Hungary with undifferentiated nonkeratinizing nasopharyngeal carcinoma (NPC). She presented to the ophthalmology clinic with no light perception in her right eye. She reported a 2-month history of right eye protrusion and swelling, right-sided hearing loss, and right temporal pain. Her past medical history was significant for a hysterectomy. She did not take any medications and did not have any drug allergies. There was no family history of head or neck cancer. She was a nonsmoker and did not have a history of radiation exposure.

Her visual acuity (VA) was no light perception OD and 20/20 OS. She had a grade 3+ right relative afferent pupillary defect. She had approximately 50% restriction in supraduction of her right eye, and her other extraocular movements were full. She had 7 mm of proptosis of her right eye with resistance to retropulsion. Slit lamp examination was within normal limits. Dilated funduscopic exam showed mild superior right disc edema with minimal pallor.

A computed tomography scan of the orbits showed a mildly homogenous enhancing mass centred within the right superior orbital fissure measuring up to 5.5 × 2.3 × 2.4 cm (Fig. 1A, B). There was expansion into the right superior orbital fissure with no frank bony destruction. The mass extended along the right optic foramen to the right cavernous sinus, which displaced the right optic nerve superiorly. The mass also partially abutted the right medial, lateral, and inferior rectus muscles, resulting in anterior displacement of the right globe. No frank invasion of the globe was seen. There was extension of the mass into the right parapharyngeal space, pterygopalatine fossa, and infratemporal fossa. Superiorly, the tumour extended into the right sphenoid sinus and along the right vidian canal and foramen rotundum.

An otolaryngology consultation was obtained and otoscopic and endoscopic examinations were performed. The patient’s oral cavity was unremarkable. Otoscopy showed a right middle ear effusion. Palpation of the neck revealed a palpable, mobile, and firm 4-cm lymph node in the right level II region. Endoscopic examination revealed a large exophytic mass within the nasopharynx, centred in the right fossa of Rosenmuller. The remainder of the fibre-optic examination was unremarkable, including the oropharynx and larynx.

A transnasal biopsy using a 0-degree endoscope and topical anesthesia was performed. The specimen was sent in formalin along with Epstein–Barr virus-encoded ribonucleic acid (EBER) in situ hybridization and a separate specimen for lymphoma protocol. The final pathology revealed a nonkeratinizing, undifferentiated NPC composed of cells with round, ovoid, and pleomorphic nuclei. The malignant cells showed prominent nucleoli present in an indistinct eosinophilic cytoplasm, growing in a syncytial pattern in a dense lymphoplasmacytic infiltrate. Immunohistochemical staining was positive in the tumour cells for keratins (pan keratin AE1/AE3, K903, and CK5) and p63. Staining for CD45 was positive in inflammatory cells in the lesion, and staining for CD3 and CD20 were positive in lymphocytes in the lesion with negative staining for CD30. No evidence of B-cell lymphoproliferative disorder was seen. In situ hybridization for EBER was positive in the tumour cells. The patient elected to return to Hungary for further investigations and treatment.

NPC is a relatively rare cancer that has a higher incidence in southern China, southeast Asia, northern Africa, and Alaska. NPC arises in the epithelial lining of the nasopharynx and is commonly associated with the presence of Epstein–Barr virus. The World Health Organization histologic classification of NPC has 3 categories: keratinizing squamous cell carcinoma (type I), differentiated nonkeratinizing carcinoma (type 2), and undifferentiated nonkeratinizing carcinoma (type 3). Patients with NPC can present with a variety of symptoms including neck mass, nasal and aural dysfunction, headache, diplopia, facial numbness, weight loss, and trismus.^,  A retrospective analysis of 4768 patients with undifferentiated or nonkeratinizing NPC identified that a neck mass (76%) was the most common presenting symptom, followed by ipsilateral nasal obstruction (73%) and aural dysfunction (62%). Cranial nerve palsies were found in 20% of patients with NPC. When a cranial nerve palsy is present, the trigeminal nerve (12.5%) or abducens nerve (10.5%) are typically involved, although involvement of each cranial nerve has been reported. Optic nerve involvement, with subsequent decreased VA, is a very rare presentation of NPC. There have been only 6 reports of decreased VA as the initial presenting feature of NPC, in either the primary or recurrent setting, in which only 2 of these cases presented with no light perception vision loss.^{,  ,  ,  ,  ,}  In contrast to the previously published case reports, this patient was from Hungary, an area not generally recognized as having a high incidence of NPC. This case further highlights that ophthalmic signs and symptoms may occasionally be the initial manifestation of NPC.