Journal of Neuro-Ophthalmology, June 2010, Volume 30, Issue 2

Nasopharyngeal Carcinoma Presenting With Rapidly Progressive Severe Binocular Optic Neuropathy and Periocular Painina Young Man

A 27-year-old man presented with rapid and severe visual loss in both eyes, together with pain behind the eyes. Visual acuities were light perception in both eyes. Pupillary constriction to light was minimal, and ophthalmoscopy results were normal. For a presumptive diagnosis of retrobulbar optic neuritis, he was treated with intravenous corticosteroids, and vision improved transiently. But vision later worsened to no light perception, and MRI revealed bilateral optic nerve enhancement with dural enhancement and thickening in the anterior skull base, sella, and retroclival areas, findings initially interpreted as inflammatory. Nasopharyngoscopy disclosed a soft tissue lesion filling the apex of the nasopharynx and the posterior portion of the ethmoid sinus with associated sinusitis. Biopsy demonstrated a moderately differentiated squamous cell carcinoma believed to have originated in the nasopharynx. This is the first case of bilateral severe optic neuropathy in nasopharyngeal carcinoma invading the skull base. It is reported to emphasize that rapidly progressive severe bilateral optic neuropathy in a young patient with periocular pain need not be caused by inflammation.

Nasopharyngeal Carcinoma Presenting With Rapidly Progressive Severe Binocular Optic Neuropathy and Periocular Pain in a Young Man Kyung-Ah Park, MD, Sei Yeul Oh, MD Abstract: A 27-year-old man presented with rapid and severe visual loss in both eyes, together with pain behind the eyes. Visual acuities were light perception in both eyes. Pupillary constriction to light was minimal, and ophthalmoscopy results were normal. For a presumptive diagnosis of retrobulbar optic neuritis, he was treated with intravenous corticosteroids, and vision improved transiently. But vision later worsened to no light percep-tion, and MRI revealed bilateral optic nerve enhancement with dural enhancement and thickening in the anterior skull base, sella, and retroclival areas, findings initially interpreted as inflammatory. Nasopharyngoscopy dis-closed a soft tissue lesion filling the apex of the naso-pharynx and the posterior portion of the ethmoid sinus with associated sinusitis. Biopsy demonstrated a moder-ately differentiated squamous cell carcinoma believed to have originated in the nasopharynx. This is the first case of bilateral severe optic neuropathy in nasopharyngeal carcinoma invading the skull base. It is reported to emphasize that rapidly progressive severe bilateral optic neuropathy in a young patient with periocular pain need not be caused by inflammation. Journal of Neuro-Ophthalmology 2010;30:150-152 doi: 10.1097/WNO.0b013e3181ce2ce7 2010 by North American Neuro-Ophthalmology Society Nasopharyngeal carcinoma rarely affects the optic nerves; only 5 cases have been described (1-5). We report a patient with carcinoma who presented with rapidly progressive, severe bilateral visual loss and periocular pain as the initial symptoms. CASE REPORT A 27-year-old man was referred to our clinic with a 3-day history of declining vision in both eyes and pain behind his eyes. One year previously, vision was 20/20 in both eyes. The patient had no contributory past medical history. Examination elsewhere disclosed only light perception in both eyes. For a diagnosis of optic neuritis, he was treated with 1 g intravenous methylprednisone daily for 3 days. Visual acuity initially improved to counting fingers but worsened several days later. On our examination, the patient could only perceive light in both eyes. Pupils reacted slowly and weakly to light without a relative afferent pupillary defect. Results of the ocular motility examination were normal. The anterior oc-ular segments and fundi were normal in both eyes. MRI delineated bilateral optic nerve and dural enhance-ment and thickening in the anterior skull base, sella, and retroclival area (Fig. 1). There was no demonstrable mass lesion compressing the optic nerves. T2 imaging demon-strated a low-signal-intensity soft tissue lesion filling the posterior portion of the ethmoid sinus with associated active inflammation in both the ethmoid and sphenoid sinuses. CT demonstrated a soft tissue lesion in the apex of the naso-pharynx. There was no bony destruction around the optic canal (Fig. 1). The imaging findings were initially interpreted as indicating sinusitis or an idiopathic inflammatory lesion (‘‘pseudotumor'') involving the sinuses, optic nerves, sella, and skull base. Nasopharyngoscopic examination disclosed a pink-purple, edematous nasal septum and an ulcerofungating nasopharyngeal mass. Endoscopic biopsy of the nasal mucosa and the sinus wall demonstrated that the mass was a moderately differentiated squamous cell carcinoma (Fig. 2). The patient was referred for chemotherapy. DISCUSSION Nasopharyngeal carcinoma is common in southern China, Hong Kong, and Singapore, with the incidence being much higher among Chinese than among Caucasians (6,7). This carcinoma commonly presents with a neck mass, blood- Department of Ophthalmology, Samsung Medical Center, Sung-kyunkwan University School of Medicine, Seoul, Korea. Address correspondence to Sei Yeul Oh, MD, Department of Ophthalmology, Samsung Medical Center, Ilwon-dong, Kangnam-gu, Seoul 135-710, Korea; E-mail: [e-mail] 150 Park and Oh: J Neuro-Ophthalmol 2010; 30: 150-152 Original Contribution Copyright © North American Neuro-ophthalmology Society.Unauthorized reproduction of this article is prohibited. tinged sputum or rhinorrhea, headache, diplopia, hearing loss, or facial pain (8). Although ophthalmic involvement is not uncommon in patients with nasopharyngeal carcinoma in the late stages of disease, isolated optic nerve involvement is rarely reported as an initial manifestation (1-5). In our patient, there was sequential bilateral visual loss over the course of 3 days, accompanied by retrobulbar pain. Among previous reports of nasopharyngeal carcinoma ini-tially presenting as visual loss, pain was an associated symptom in 3 patients (2,4,5), and no pain or discomfort was reported in another 3 patients (1,3). In all previous patients, the visual loss was monocular (1-5). In the current report, bilateral and nearly simultaneous blindness was the initial presentation of nasopharyngeal carcinoma, suggest-ing a rapidly progressive condition. The blindness in nasopharyngeal cancer is due to optic nerve compression from direct extension of the tumor (1). Four previously reported patients demonstrated a mass lesion around the optic nerve (1,3,4), but there was no demonstrable mass lesion in 2 other patients, and the au-thors suggested a paraneoplastic effect (2,5). In the current report, direct compression of the optic nerve by the tumor was not obvious, but the optic nerves enhanced as did contiguous regions. Whether the enhancement is due to inflammation or direct infiltration of the optic nerve sheath is uncertain. FIG. 1. Precontrast (A) and postcontrast (B) T1 axial MRI shows enhancing tumor infiltrating the skull base (asterisk) and enhancing optic nerve sheaths bilaterally (short arrows). C. Postcontrast T1 coronal MRI shows enhancement of both optic nerves (short arrows), the sellar area (long arrow), and the mucosal wall of the sphenoid sinus (arrowheads). D. Postcontrast T1 sagittal MRI shows sellar enhancement (arrow), dural thickening and enhancement in the retroclival area (arrowheads), and irregular enhancement of the mucosal wall of the sphenoid sinus (asterisks). E. Axial CT reveals a soft tissue lesion (asterisk) in the apex of the nasopharynx. F. Coronal CT at the bone window setting demonstrates no bone destruction. Original Contribution Park and Oh: J Neuro-Ophthalmol 2010; 30: 150-152 151 Copyright © North American Neuro-ophthalmology Society.Unauthorized reproduction of this article is prohibited. Our patient represents the youngest reported case of optic neuropathy in nasopharyngeal cancer (1-5), although 2 previously reported cases involved patients in their early 30s (2,4). Clinicians usually suspect an inflammatory disorder rather than a malignant process in patients of this age. In 3 previously reported patients (2,4,5), including these relatively young patients, optic neuritis was the initial diagnosis, and corticosteroid treatment was initiated. Transient improvement in vision after steroid therapy de-layed the correct diagnosis in our case as well as previous reports (2,4,5). REFERENCES 1. Carlin L, Biller J, Laster DW, Toole JF. Monocular blindness in nasopharyngeal cancer. Arch Neurol. 1981;38:600. 2. Hoh ST, Teh M, Chew SJ. Paraneoplastic optic neuropathy in nasopharyngeal carcinoma-report of a case. SingaporeMed J. 1991;32:170-173. 3. Kao LY, Chuang HC, Liang YS. Visual loss as the initial presentation of nasopharyngeal carcinoma. J Clin Neuroophthalmol. 1993;13:24-26. 4. Prasad U, Doraisamy S. Optic nerve involvement in nasopharyngeal carcinoma. Eur J Surg Oncol. 1991;17: 536-540. 5. Tsai CC, Ho HC, Kau HC, Kao SC, Hsu WM. Optic neuritis: a rare manifestation of nasopharyngeal carcinoma. Eye. 2002;16:501-503. 6. Hughes PJ, Scott PM, Kew J, Cheung DM, Leung SF, Ahuja AT, van Hasselt CA. Dysphagia in treated nasopharyngeal cancer. Head Neck. 2000;22:393-397. 7. Witte MC, Neel III HB. Nasopharyngeal Cancer. Baltimore: Lippincott-Raven; 1998:1637-1652. 8. Hsu MM, Tu SM. Nasopharyngeal carcinoma in Taiwan: clinical manifestations and results of therapy. Cancer. 1983; 52:362-368. FIG. 2. Histopathologic examination of biopsy specimen reveals nests of atypical squamous cells consistent with moderately differentiated squamous cell carcinoma. (Hematoxylin and eosin, 3100). Original Contribution 152 Park and Oh: J Neuro-Ophthalmol 2010; 30: 150-152 Copyright © North American Neuro-ophthalmology Society.Unauthorized reproduction of this article is prohibited.