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Show Journal of Neuro- Ophthalmology 20( 1): 20- 21, 2000. i 2000 Lippincott Williams & Wilkins, Inc., Philadelphia Third Nerve Palsy as the Presenting Manifestation of Esthesioneuroblastoma Andrew G. Lee, MD, and Rosa A. Tang, MD A 52- year- old patient experienced unilateral third nerve palsy because of cavernous sinus involvement of biopsy- proven esthesioneuroblastoma. The patient experienced resolution of diplopia after surgical debulking of the tumor. Clinicians should be aware of the neuro- ophthalmologic manifestations of esthesioneuroblastoma. Key Words: Esthesioneuroblastoma- Third nerve palsy. Esthesioneuroblastoma ( ENB) is an uncommon tumor arising from olfactory neural crest epithelium within the nasal cavity. Neuro- ophthalmologic manifestations and intracranial involvement have been infrequently described ( 6- 8,10). We report a 52- year- old man presenting with a unilateral third nerve palsy because of cavernous sinus involvement of biopsy- proven ENB. The patient experienced resolution of diplopia following surgical debulking of the tumor. Esthesioneuroblastoma ( ENB) is an uncommon tumor that usually arises from olfactory neural crest epithelium within the nasal cavity. The most common clinical presentation of ENB is unilateral persistent nasal obstruction, but other otolaryngologic symptoms and signs include epistaxis, rhinorhea, pain, and anosmia ( 1- 4,6). Although ophthalmologic manifestations of ENB have been reported previously related to orbital involvement ( e. g., proptosis, ophthalmoplegia), neuro- ophthalmologic manifestations and intracranial involvement have been infrequently described ( 7- 10). We report a patient presenting with a third nerve palsy because of intracranial involvement of ENB. CASE REPORT A 52- year- old man sought treatment in October 1995 after a 3- day history of left- sided ptosis, diplopia, left nasal bleeding, and left frontal headache. Medical history was significant for hypopituitarism treated with thyroid supplementation, hydrocortisone, and testosterone intramuscular injections. He had not undergone neuroimaging This work was supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, NY. Manuscript received August 12, 1998; accepted October 13, 1999. From the Departments of Ophthalmology, Neurology, and Neurosurgery ( AGL), Baylor College of Medicine, Houston, Texas, and the Department of Neurosurgery, M. D. Anderson Cancer Center and the University of Texas, Houston, Texas; and the Department of Ophthalmology at the University of Texas Medical Branch at Galveston ( RAT), Galveston, Texas. Dr. Lee is now with the Department of Ophthalmology, The University of Iowa Hospital and Clinics, Iowa City, Iowa. Address correspondence and reprint requests to Andrew G. Lee, MD, 200 Hawkins Drive, University of Iowa Department of Ophthalmology, Iowa City, IA 52242. or evaluation for his hypopituitarism before presentation in October 1995. He had chronic sinusitis treated with oral decongestants. The remainder of his medical, surgical, ocular, social, and family history was noncontribu-tory. Ophthalmologic examination revealed a complete ptosis OS and complete elevation, depression, and adduction deficit OS consistent with a left third cranial nerve palsy. Abduction of the OS was normal. The OD moved normally. The right pupil measured 4 mm and the left pupil measured 4.5 mm. Both pupils reacted well to light, and there was no relative afferent pupillary defect. Corneal sensation was intact bilaterally. Ophthalmoscopy was normal in each eye. A magnetic resonance imaging scan of the head revealed a 4- cm oval mass in the left sphenoid sinus extending into the prepontine cistern, sellar area, cavernous sinus, and dorsum sellae ( Fig. 1). There was no evidence of orbital involvement. A computed tomography scan of the sinuses revealed sinusitis in the ethmoid air cells and maxillary antrum bilaterally. Left sphenoidotomy, widening of the maxillary ostia, and left anterior and posterior ethmoidectomy were performed. Pathology from the left sphenoid sinus revealed neoplastic cells that exhibited positive immu-nohistochemic staining for synaptophysin. Histochemi-cal stains were negative for S- 100, leukocyte common antigen, cytokeratin, vimentin, and chromogranin, and the pathology was felt to be consistent with esthesioneuroblastoma. The patient declined to undergo postoperative chemotherapy or radiation therapy. Over the next several months, he had complete resolution of the left third nerve palsy. A computed tomography scan 3 months later revealed residual soft- tissue mass within the sphenoid sinus and erosion into the left cavernous sinus. Ophthalmologic examination 18 months after the initial onset of symptoms revealed a visual acuity of 20/ 20 in each eye. The extraocular motility exam was normal. There was no ptosis. The pupils were equal in size and reacted normally to light. Ophthalmoscopy was normal in each eye. No further follow- up or neuroimaging is available. DISCUSSION The ophthalmologic manifestations of ENB include periorbital pain, epiphora, lid edema, proptosis, conjunctival chemosis and injection, ptosis, ophthalmoplegia, and optic atrophy ( 1- 10). Rakes et al. ( 6) reported ocular signs and symptoms in 53% ( 20 out of 38) of patients at the Mayo Clinic upon initial presentation. The most common ocular problems were periorbital pain ( 24%) and excessive tearing ( 21%). Two patients had papilledema, 20 ESTHESIONEUROBLASTOMA 21 FIG. 1. A: Axial, noncontrast enhanced T1- weighted magnetic resonance imaging scan demonstrates isointense signal intensity within the cavernous sinus on the left ( arrow). B: Coronal T1 - weighted contrast magnetic resonance imaging scan of the head demonstrates heterogeneous signal intensity and contrast enhancement within the left sphenoid sinus. i r > > i 9 three patients had ptosis, and three patients developed cranial neuropathies. Rakes et al. ( 6) described two patients in detail, one of whom had diplopia, proptosis, and blurred vision because of orbital involvement. Dulgerov and Calceterra ( 2) reported extraocular muscle paralysis in 4% of 26 patients with ENB. Tucker et al. ( 9) reported a 64- year- old woman with left trigeminal hypoesthesia, bilateral visual loss, right optic atrophy, and ophthalmoplegia. The patient was treated with orbital radiotherapy, surgical resection, and chemotherapy, but she died from recurrent disease. Berman et al. ( 1) reported an 11- year- old girl with ENB presenting as sudden unilateral blindness because of a compressive optic neuropathy. The left optic nerve was compromised by lateral expansion of the ENB from the sphenoid sinus into the optic canal and medial left orbit. Goldhammer et al. ( 3) reported a case of intracranial ENB with unilateral visual loss and reviewed nine other cases from the literature of intracranial involvement. Of these ten cases, three ( 30%) had visual loss because of involvement of the optic nerve resulting from tumor expansion ( 3). Rodas et al. ( 7) in 1986 reported a case of intracranial metastasis because of ENB to the right parietal cortex. In addition, this patient suffered an attack of acute angle closure glaucoma and an ischemic optic neuropathy that were both attributed to radiation therapy ( 6500 rads to the left nasal cavity and 4950 rads to the anterior cervical area). Hamilton et al. ( 4) reported an 18- year- old man with ENB who developed a right optic neuropathy, right ptosis, and elevation deficit because of an intracranial ENB with extension into the right superior orbit. Wilson ( 10) reported a 35- year- old man with anosmia and acute visual loss because of a compressive left optic neuropathy. There was extension of ENB into the orbit and anterior cranial fossa surrounding the optic nerve. Our patient presented the unusual finding of an oculomotor cranial neuropathy that subsequently resolved following surgical decompression of the left sphenoid sinus. The microscopic features of ENB usually include smooth, contoured cellular nests within a vascular stroma or less commonly diffuse sheet- like growths supported by delicate capillaries. Neuronal markers have also been helpful in defining these tumors. The treatment of ENB usually requires extensive craniofacial surgical resection, and most authors recommend adjunctive radiotherapy ( 1^ 4,6- 9). In addition, chemotherapy has been employed with some success. Our patient underwent resection without chemotherapy or radiotherapy. Local recurrence of ENB is common ( 48%), and metastatic disease occurs in 20% to 63% of cases ( most commonly involve the lymph nodes or lungs). Leptomenin-geal involvement and intracranial metastatic disease are uncommon in ENB. Dulgeurov and Calcaterra ( 2) reviewed 26 cases, of which 74% patients were alive at 5 years and 60% were alive at 10 years. Combined treatment with radiotherapy and surgery was recommended, based upon a recurrence- free status of 92% in patients treated with combination therapy, compared with surgery alone ( 14%) or radiation alone ( 40%). Clinicians should be aware of the ophthalmologic manifestations of ENB, including proptosis, ophthalmoplegia, and an optic neuropathy. Although uncommon, neuro- ophthalmologic findings may occur because of intracranial extension of tumor. REFERENCES 1. Berman EL, Chu A, Wirtschaffter JD, et al. Esthesioneuroblastoma presenting as sudden unilateral blindness. Histopathologic confirmation of optic nerve demyelination. J Clin Neuro- ophthalmol 1992; 12: 31- 6. 2. Dulgerov P, Calcaterra T. Esthesioneuroblastoma: the UCLA experience 1970- 1990. Laryngoscope 1992; 102: 843- 9. 3. Goldhammer Y, Sadeh M, Tadmor R, Leventon G. Intracranial esthesioneuroblastoma associated with unilateral visual loss. Case report. J Neurosurg 1980; 53: 836- 40. 4. Hamilton AE, Rubinstein LJ, Poole GJ. Primary intracranial esthesioneuroblastoma ( olfactory neuroblastoma). J Neurosurg 1973; 38: 548- 56. 5. Johnson LN, Krohel GB, Yeon EB, Pames SM. Sinus tumors invading the orbit. Ophthalmology 1984; 91: 209- 17. 6. Rakes SM, Yeatts RP, Campbell RJ. Ophthalmic manifestations of esthesioneuroblastoma. Ophthalmology 1985; 92: 1749- 1753. 7. Rodas RA, Erkman- Balis B, Cahill DW. Late intracranial metastasis from esthesioneuroblastoma: case report and review of the literature. Neurosurgery 1986; 19: 622- 7. 8. Schochet SS, Jr, Peters B, O'Neal J, et al. Intracranial esthesioneuroblastoma. A light and electron microscopic study. Acta Neu-ropathol 1975; 31: 181- 9. 9. Tucker SM, Konrad HR, Wacaser L. Esthesioneuroblastoma with orbital involvement. Ann Ophthalmol 1997; 29: 326- 8. 10. Wilson WMG, Cullen G. Olfactory groove tumor causing blindness: a case report. Can J Ophthalmol 1967; 2: 622- 7. J Neuro- Ophlhalmol, Vol. 20, No. 1, 2000 |
