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Show J. Clin. Neuro-ophthalmol. 4: 259-263, 1984. Neurilemmoma of the FrontalEthmoid Sinus Simulating a Mucocele A Case Report JONATHAN D. CHRISTENBURY, M.D. HOWARD N. SHORT, M.D. ALFRED G. WINDHAM, M.D. Abstract A 48-year-old black female had a I-year history of increasing proptosis and headache. Sinus films and CT scan demonstrated an expansive soft tissue mass in the right frontal-ethmoid sinus with lateral displacement of the medial orbital wall, consistent with a mucocele. Resection revealed a solid tumor which was identified pathologically as a neurilemmoma. To our knowledge, this is the first published report of a frontal-ethmoid neurilemmoma presenting solely with proptosis, and also mimicking a mucocele. Neurilemmomas of the paranasal sinuses are uncommon tumors, and previously reported patients have had nasal symptoms. This is the first published report of a neurilemmoma of a paranasal sinus simulating a mucocele, with unilateral proptosis as the only symptom. Case Report A 48-year-old black female noticed increased swelling~around the right eye for approximately 1 year, and experienced intermittent dull headaches localized to the right frontal region. There were no associated visual or nasal symptoms. There was no other significant medical history. Best-corrected vision was 20/20 -3 in the right eye and 20/20 in the left eye. Pupils were equally reactive and there was no afferent defect. External exam revealed 2 mm of proptosis of the right eye with displacement downward and laterally. A movable and somewhat rubbery mass was pal- From the Departments of Ophthalmology (JOe. HNS) and Otolaryngologv (AGW). Duke University Medical Center. Durham. North Carolina pable in the superior nasal quadrant of the right orbit. Extraocular movements were normal and there was no diplopia. Slit lamp examination, dilated fundus examination, and Goldmann kinetic visual fields were within normal limits. Examination of the nasopharynx failed to demonstrate a mass. The patient had no stigmata of neurofibromatosis. Radiologic studies included sinus films, orbital tomograms, and orbital CT scan. Sinus films (Figs. la and 1/1) demonstrated an expansile soft tissue mass in the right fronto-ethmoid sinus crossing into the left fronto-ethmoid complex, as well as lateral displacement of the superomedial orbital wall. Orbital tomograms confirmed the soft tissue expansion and displacement of the medial orbital wall. Coronal and axial CT scans demonstrated a soft tissue mass in the right ethmoid sinus with bowing of the lamella papyracae laterally into the right orbit (Fig. 2a), and bowing of the frontal bone into the anterior cranial fossa (Fig. 2/1). The radiologic diagnosis was consistent with a mucocele. The patient underwent a right fronto-ethmoidectomy. At the time of operation, it was noted that the medial wall of the orbit was displaced laterally approximately 1 cm, with a dehiscence in the floor of the frontal bone. A solid, encapsulated mass \vas found to fill the entire right anterior and posterior ethmoid and frontal sinuses and was removed piecemeal. The mass originated from a stalk protruding through a small dehiscence just laterally to the anterior portion of the right cribiform plate. Histopathologic examination of the tumor demonstrated whorls of spindle-shaped cells (Fig. 3a). The nuclei were long and narrow with finely granular chromatin, often demonstrating "palisading," characteristic of a neurilemmoma (Fig. 3/J ). A thick fibrous capsule surrounded the tumor. The stalk contained nerve and vascular tissue. 259 Figures la and lb. Plain sinus films showing soft tissue density in the right frontoethmoid CL>mple~ (tI). and lateral displacement of the right medial orbital wall (/1). 260 Journal uf Clinical Neuro-ophlhalmology Christenbury, Short, Windham ~b\ Figures 2a and 2b. Enhanced axial CT scan demonstratmg soft tissue expansive mass in right ethmoid sinus displacing medial wall of orbit, medial rectus, and globe (a), and extension into the right frontal sinus (/1) December 1984 261 Neurilemmoma figures 30 and 3b. I'hUl<lmicwgr<\ph dl'mlmstrating ",hurls uf spindlt'-shapt'd ct'!ls (til, with bt'nign cytology and ·palisading· {Ill (Hl'm.lll1wlin-l',,,in. Original magnificatilll\ X100 and x-lOO) 262 Journal oi Clinical Neuro-ophthalmology Discussion To our knowledge, this is the first published report of a fronto-ethmoid neurilemmoma clinically simulating a mucocele; and the second report of a neurilemmoma involving the frontal sinus] Although solitary neurilemmomas of the head and neck comprise one-third of the incidence of this tumor, involvement of the paranasal sinuses is uncommon. Kragh et al. 2 reported 143 patients in which five cases involved the nasal fossa, including one ethmoid tumor involving the medial orbit with a proptosis of the globe. However, all five of these patients presented with nasal symptoms. In other series, 3-5 nasal obstruction, epistaxis, and facial swelling were the most common symptoms, often associated with proptosis. Nasal-ethmoid involvement is most frequent followed by maxillary, intranasal, and sphenoid sinus. Tumors arising from the sphenoid sinus may cause visual loss, cranial nerve III, IV, and VI deficits, and deep orbital or cranial pain." Only one other neurilemmoma of the frontal sinus has been reported and presented with eNS findings and pneumocephalus. 1 Neurilemmomas are benign tumors of the Schwann cells and may arise from any of the peripheral, autonomic, or cranial nerves excluding the optic and olfactory nerves. They are slowgrowing and reach large size before compromising the nerve from which they originate. Neurilemmomas are often multiple in neurofibromatosis and rarely may be malignant.5 Histologically, the tumor is encapsulated, exhibits varying degrees of cellularity with benign-appearing cytology and may show myxoid degeneration. Blood vessels often show thickening and hyalinization. Neurilemmomas are fairly radio-resistent and the treatment of choice is st.i'rgical excision of the tumor within its capsule. Specific operative approaches depend upon the location of the tumor and have been discussed.' A number of lesions occurring in the paranasal sinuses may be difficult to distinguish clinically from mucoceles. H13 Although certainly a rare ent- December 1984 Christenbury, Short, Windham ity, neurilemmoma, as well as other neoplastic processes, should be considered in the differential diagnosis of expanding mass lesions of the paranasaI sinuses. References 1. Miglets, AW., Rood, L and Lucus, J.G.: Pneumocephalus from a frontal-ethmoid neurilemmoma. Arc//. Otolan/llgo/. 109: 417-419,1983. 2. Kragh, L.V., Soule, E.H., and Masson, J.K.: Benign and malignant neurilemmomas of the head and neck. Sllrg. GYllecol. Obstet. 107: 211-218, 1960. 3. Iwamura, S., Sugiura, S., and Momura, Y: Schwannoma of the nasal cavity. Arch. Oto/aryllgol. 96: 176-177, 1972. 4. Robitaille, Y, Seemayer, T.A., and Deiry, A.E.: Peripheral nerve tumors involving paranasal sinuses: A case report and review of the literature. Callcer 35: 1254-1258, 1975. 5. Perzin, KH, Panyu, H, and Wechter, S.: Nonepithelial tumors o( the nasal cavity, paranasal sinuses and nasopharynx. Callcer 50: 2193-2202, 1982 6. Calcaterra, T.e., Rich, R, and Ward, P.W.: Neurilemmoma of the sphenoid sinus. Arch. Otolan/Ilgol. 100: 383-385, 1974. 7. Shugar, M.A., Montgomery, W.W., and Reardon, E.J.: Management of paranasal sinus schwannoma. Alii/. Otolan/llgo/91: 65-69, 1982. 8. Mortada, A.: Dermoid cvst of great wing of sphenoid bone. Br. f. Ophthall11ol. 54: 131-133, 1970. 9. Cullen, J.F.: Orbital diplopic dermoids. Br. f. Ophthall11ol. 58: 105-106, 1970. 10. Pollock, J., Newton, T., and Hoyt, W.: Transsphenoidal and ethmoid encephalocele. Arch. Ophthalmol. 83: 445-447, 1970 11. Mohan, H., and Sen, O.K.: Orbital affection in nasal and paranasal neoplasms. Acta Ophtha/mol. 47: 289-294, 1969. 12. Brownson, R.J.: Adenocarcinomas of the prostate presenting as a frontal sinus mucocele. Lan/Ilgoscope 79: 458-469, 1969. 13. Guerry, RK., and Smith, JL.: Paranasal sinus carcinom'a causing orbital mucocele. Am. J. Ophtha/1110180: 943-946,1975 Write for reprillts to: Jonathan D. Christenbury, M.D., Duke University Eye Center, Box 3802, Durham, North Carolina 277l(). . 263 |
