| OCR Text |
Show ORIGINAL CONTRIBUTION Papilledema as the Presenting Manifestation of Spinal Schwannoma Fiona Costello, MD, Randy H. Kardon, MD, PhD, Michael Wall, MD, Patricia Kirby, MD, Timothy Ryken, MD, and Andrew G. Lee, MD A 63- year- old woman with headache, blurred vision, bilateral optic disc edema, and normal cranial magnetic resonance imaging scan underwent lumbar puncture that revealed an elevated opening pressure ( 290 mm water), a protein level of 114 mg/ dl, and mild pleocytosis. Spinal magnetic resonance imaging later demonstrated a sacral tumor, which proved to be a schwannoma with sarcoid- like features. After surgical removal of the tumor, the patient's manifestations resolved. This case emphasizes that low spinal cord tumors can cause elevated intracranial pressure without causing markedly elevated cerebrospinal fluid protein or cells, or any myelopathic manifestations, perhaps by obstructing sacral cerebrospinal drainage. Comprehensive spine imaging should be a part of the evaluation of a patient with papilledema who has normal brain imaging but abnormal spinal fluid constituents. ( JNeuro- Ophthalmol 2002; 22: 199- 203) Spinal tumors are an uncommon cause of increased intracranial pressure ( 1- 8). Papilledema may be the sole clinical manifestation ( 1,2,6). We present a case of a sacral schwannoma that caused papilledema without symptoms of spinal dysfunction, and discuss the reported tumor types and locations, as well as possible mechanisms for increased intracranial pressure. CASE REPORT A 63- year- old woman came for treatment because of a 3- month history of intermittent blurred vision and occasional headaches. She did not describe experiencing pulse-synchronous tinnitus or double vision. Her medical history included diabetes, hypothyroidism, controlled hyperten- Departments of Ophthalmology ( FC, AGL, RHK, MW), Neurology ( AGL, MW), Pathology ( PK), and Neurosurgery ( AGL, TR), University of Iowa Hospitals and Clinics, Iowa City, Iowa. Supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, NY. Address correspondence to Andrew G. Lee, MD, Department of Ophthalmology, 200 Hawkins Drive PFP, University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA; E- mail: andrew- lee@ uiowa. edu sion, hyperlipidemia, and remote seizures. Her regular medications were Lipitor, Glucophage, Synthroid, and multivitamins; She did not describe using lithium, tetracycline, vitamin A, or corticosteroids. Her weight had been stable in the previous year. The patient's height was 5' 1", and her weight was 200 pounds. The results of a general neurologic examination were completely normal. The ophthalmology evaluation revealed a best- corrected visual acuity of 20/ 25 OD and 20/ 20 OS. Slit lamp biomicroscopy showed nuclear cataract. The pupils, intraocular pressures, and ocular motility were normal. Ophthalmoscopy showed bilateral optic disc edema with surrounding peripapillary hemorrhages ( Fig. 1). Goldmann perimetry showed enlargement of the blind spots in both eyes ( Fig. 2). Magnetic resonance imaging ( MRI) scans of the head were normal except for dilation of both optic nerve sheaths and flattening of the posterior globes in both orbits; these findings were consistent with raised intracranial pressure. The sella turcica was normal. A lumbar puncture revealed an opening pressure of 290 mm water. Analysis of the cerebrospinal fluid demonstrated an elevated protein of 114 mg/ dl ( normal < 46 mg/ dl) and 20 leukocytes mm3 ( normal < 5 leukocytes), 98% of which were monocytes. The CSF formula was otherwise normal, as were urinalysis and baseline serologic studies. Magnetic resonance imaging was performed along the entire spinal axis in search of a source of FIG. 1. Fundus, showing optic disc edema and peripapillary hemorrhages. „ JNeuro- QpMhalxnol,. Vol. 22,% No~ 3, 2002 „ , „ , . , . , . , DOI: 10.1097/ 01. WNO. 00000288.62.11.084. C3 , .199 , Copyright © Lip pincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 3, 2002 Costello et al. FIG. 2. Goldmann perimetry, showing enlarged blind spots. meningeal inflammation. It revealed an ovoid, soft tissue mass within the thecal sac at the S1- S2 disc space ( Fig. 3 A), measuring 1.7 cm craniocaudally, 1 cm anteroposteriorly, and 1 cm transversely. The lesion enhanced intensely and homogeneously ( Fig. 3B). Subtle enhancement was also noted along both the anterior and posterior aspects of the distal spinal cord near the conus medullaris. No other enhancing lesions were found within the spinal cord. An incidental hemangioma was demonstrated within the Tl 1 vertebral body, but the marrow signal was otherwise normal throughout the spine. On the basis of the appearance of the lesion, the diagnosis of drop metastases or filum terminale ependymoma was considered. Se: 16 Im: 6 Sag RIO. 8 FIG. 3. ( A) Sagittal T2- weighted magnetic resonance imaging scan of lumbosacral spine shows Tl 1 vertebral body hemangioma ( open arrow) and soft tissue mass within the thecal sac at S1- S2 ( closed arrow). ( B) Sagittal enhanced Tl - weighted magnetic resonance imaging scan of lumbosacral spine showing Tl 1 vertebral body hemangioma ( open arrow) and enhancement of the thecal mass at S1- S2 ( closed arrow). „ 200 . , „.. , „ . . © 2002 Lippincott Williams & Wilkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. PAPILLEDEMA AND SPINAL SCHWANNOMA JNeuro- Ophthalmol, Vol. 22, No. 3, 2002 The patient underwent L5 and SI laminectomies, intradural tumor exploration, excision, and microscopic exploration. Histopathology showed myxoid lobules of spindle and oval cells, without atypia, arranged in fascicles and whorls, with no increased mitotic activity ( Fig. 4A). Immunohistochemistry showed positive staining with SI00, but keratin and epithelial membrane antigen stains were negative. Myxoid areas of the tumor stained with mu-cicarmine and alcian blue. Numerous large multinucleated giant cells were seen in multiple noncaseating granulomas throughout the tumor ( Fig. 4B). No organisms were seen, and stains for acid- fast bacilli and fungi were negative. The pathologic diagnosis was schwannoma with sarcoid- like reaction. Within a month of surgery, the patient had resolution of her visual symptoms and optic disc edema. In light of the sarcoid- like reaction in the tumor, several investigations were performed to exclude systemic sarcoidosis, including a chest radiograph, total body gallium scan, and serum an-giotensin- converting enzyme level ( 23 U/ L), and all results were negative. DISCUSSION Spinal tumors are an uncommon but well-documented cause of papilledema ( Table 1). This case is distinct because of the presentation with papilledema as the only clinical abnormality, and the unusual histopathologic appearance of the tumor. To our knowledge, this presentation was reported in only four cases in the English literature before 1992 ( 1,2,6). In most cases, spinal cord tumors have manifested localizing clinical signs, including back pain, weakness, and sensory loss, to suggest pathologic changes in the spine ( 1- 3,5- 7). In their review of cases reported until 1992, Matzkin et al. ( 1) noted that spinal tumors often present with lumbar pain ( 65 to 70% cases), yet those associated with papilledema are more likely to present with FIG. 4. ( A) Photomicrograph of tumor, showing palisadi multinucleated giant cell ( open arrow). ( B) Higher magnific headache, nausea, vomiting, and vision loss. Oikawa et al. ( 2) reported a case, similar to ours, of progressive vision loss and papilledema in a patient with multiple spinal neurinomas, in whom there were no spinal symptoms. In that review ( 2), 6 of 34 patients had a visual disturbance as their presenting manifestation of spinal cord tumor. Of these 6, neurologic examination disclosed that 4 had signs or symptoms of spinal dysfunction, one received a diagnosis with a spinal tumor 3 months later with pain in the right leg and back, and 1 patient had no spinal symptoms or signs. In our case, the tumor was a sacral schwannoma, a tumor noted in only 11% of the 53 cases reported by Matzkin et al. ( 1). To our knowledge, there have been no reports of spinal schwannoma with associated granulomatous reaction. Ependymoma has been the predominant spinal tumor associated with papilledema ( 40 to 50% of cases) ( 1- 8). Other less common tumors have included schwannoma ( 11%), meningioma ( 7%), neurofibroma ( 7%), and glioma ( 7%) ( 1). The finding of a sarcoid- like reaction within tumors but without clinical or radiologic evidence of systemic sarcoidosis has been previously reported ( 9), most often in the lymphatics draining a primary tumor and in the parenchyma adjacent to primary and secondary metastatic lesions ( 9). Sarcoid reaction in the parenchyma of embryonic lesions is extremely rare but has been reported in a 9- month- old child with neuroblastoma ( 9). The cause of the sarcoid reaction was not clear in that case ( 9); it may have been caused by tumor- related antigens shed by the tumor, or released by cell death. The sacral location of the spinal tumor in our case, and the associated CSF analysis findings, were consistent with other reports of papilledema- associated spinal cord tumors. Most have been located in lower spinal levels, usually in the thoracolumbar or lumbosacral regions ( 1,3- 6). Several mechanisms have been proposed to explain how such spinal tumors may increase intracranial pressure. nuclei consistent with schwannoma ( closed arrow) and a m of multinucleated giant cell ( open arrow). Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 3, 2002 Costello et al. TABLE 1. Previously reported spinal tumors manifesting with papilledema Author ( reference) Glasaeur ( 4) Raynor ( 5) Farmilo et al. ( 6) Matzkin et al. ( 1) Cases 20 37 32 54 Tumor diagnosis Ependymoma- 11 Astrocytoma- 1 Oligodendroglioma- Spongioblastoma- 1 Medulloblastoma- 1 Neurofibroma- 3 Meningioma- 1 Luetic granuloma- 1 Ependymoma- 15 Ependymoblastoma- Oligodendroglioma- Undetermined- 5 Spongioblastoma- 1 Neurinoma- 2 Medulloblastoma- 1 Neurofibroma- 3 Meningioma- 3 Astrocytoma- 1 Sarcoma- 1 Vascular lesion- 1 Granuloma- 1 Ependymoma- 17 Ependymoblastoma- Granuloma- 1 Astrocytoma- 1 Oligodendroglioma- Medulloblastoma- 1 Spongioblastoma- 1 Neuro fibroma- 2 Neuroma- 2 Neuro lemmoma- 1 Meningioma- 1 Vascular lesion- 1 40% ependymoma} 11 % schwanomma 7% meningioma 7% neurofibroma 7% glioma - 1 - 3 - 1 - 3 - 1 Tumor location Lumbar- 9 Thoracic- 7* Thoracolumbar- 5 ( 1 patient had 2 tumors) Cervical- 6 Thoracic- 7 Thoracolumbar- 25 ( 1 patient had 2 tumors) Thoracic or lumbosacral Predominantly lower thoracic or lumbar region CSF opening pressure > 150 mm water* 10/ 11 ( Not reported in 9) Not reported 14/ 15 ( Not reported in 17) Not reported Range of CSF protein ( mg/ dl) f 50- 10,500 in 15 ( Not reported in 5) > 60inl9; < 60in4 ( Not reported in 14) 38- 10,500; f > 120 in 22; < 60in3 ( Not reported in 7) 38- 10,500; > 100in66% f ( Number with normal protein not reported) Percent without spinal manifestations Not reported Not reported 4/ 14 with adequate reporting 7% * Denominator represents total cases in which measurement was reported. f The range of CSF protein in the Farmilo and Matkin series is the same because the authors reviewed the same cases. X Numbers of cases of different tumor types not available. ~ 2U2 „.. , „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. PAPILLEDEMA AND SPINAL SCHWANNOMA JNeuro- Ophthalmol, Vol. 22, No. 3, 2002 In cervical cord lesions, raised intracranial pressure has been ascribed to rostral extension and CSF flow obstruction at the level of the medullary exit foramina ( 1). This mechanism is not plausible in cases of papilledema resulting from lower spinal lesions, which represent the majority of cases. Elevated CSF protein and protein degradation products have been proposed to cause CSF flow obstruction. In the reviews to date, more than 60% of the spinal tumors associated with papilledema have been associated with elevated CSF protein levels. In the review by Matzkin et al. ( 1), the range of CSF protein was 38 mg/ dl to 10,500 mg/ dl, and greater than 100 mg/ dl in 66% of 54 cases ( our patient's CSF protein was 114 mg/ dl). It has been postulated that elevated protein may raise intracranial pressure by decreasing CSF absorption at the arachnoid villi ( 1- 8). Among the spinal tumors that cause papilledema without elevated CSF protein, the mechanism may be compromise of the lumbosacral elastic reservoir. The lower spinal location of the tumors may represent a source of mechanical obstruction to CSF flow at the level of the lumbar cul- de- sac. The lumbar portion of the spinal sac has been described as an " elastic reservoir" for CSF flow ( 1,7,8). Hence, changes in CSF volume and flow dynamics have been attributed to a reduced " decompressive" capacity in this distensible region of the spinal canal ( 1,7,8). The size of the tumor has not been a contributing factor, given that even small tumors have caused raised intracranial pressure when located within the venous plexus or intrathecal space ( 1). Other proposed mechanisms of raised intracranial pressure have included dissemination of tumor cells, production of mucinous material, secondary hemorrhage, and venous stasis with impaired CSF flow ( 1- 4,7,8). In summary, the finding of papilledema with normal cranial imaging and raised CSF protein may herald a spinal tumor, and appropriate spinal imaging is necessary to secure the diagnosis. REFERENCES 1. Matzkin DC, Slamovits TL, Genis I, et al. Disc swelling: a tall tail? Surv Ophthalmol 1992; 37: 130- 6. 2. Oikawa S, Kyoshima K, Takemae T, et al. Multiple spinal neurinomas presenting visual disturbance as the initial symptom: case report. Surg Neurol 1992; 38: 309- 14. 3. Tanaka K, Waga S, Shimosaka S. Papilledema and spinal cord tumors. Surg Neurol 1988; 29: 462- 6. 4. Glasauer FE. Thoracic and lumbar intraspinal tumours associated with increased intracranial pressure. JNeurolNeurosurgPsychiatry 1964; 27: 451- 8. 5. Raynor RB. Papilledema associated with tumors of the spinal cord. Neurology 1969; 19: 700^ 1. 6. Farmilo RW, McAuley DL, Osborne DR. Papilledema and spinal cord tumours. NZMed J 1974; 80: 100^ 1. 7. Ammerman BJ, Smith DR. Papilledema and spinal cord tumors. Surg Neurol 1975; 3: 55- 7. 8. Breen LA. Disc edema and peripheral neuropathy. Surv Ophthalmol 1994; 38: 467- 74. 9. Hojo H, Suzuki S, Kikuta A, et al. Sarcoid reaction in primary neuroblastoma: case report. Pediatr DevPathol 2000; 3: 584- 90. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. |
