Primary papillary meningioma of the optic nerve sheath: A case of unique location and benign pathology

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Primary Papillary Meningioma of the Optic Nerve Sheath : A Case of Unique Location and Benign Pathology Samruay Shuangshoti, M .D . Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand

Shuangshoti S . Primary papillary meningioma of the optic nerve sheath: A case of unique location and benign pathology . Sues Neurol 1993 ;39 :200-203 . I report here a case of primary papillary meningioma of the optic nerve sheath which developed in the left eye of a 54-year-old woman over a period of 20 years . A review of 123 primary papillary meningiomas, including the current instance, disclosed that they tended to occur in young patients of both sexes at an average age of 35 years, with one-fourth of them occurring in the first two decades of life, and with a female/male ratio of 3 : 2 . These meningiomas were most frequent in the supratentorial compartment of the cranial cavity, especially on the cerebral convexity and in the parasagittal region . They were uncommon subtentorially, intraspinally, and outside the central nervous system . Most reported papillary meningiomas were claimed to be malignant, but the tumor of this particular woman was benign pathologically and clinically . Tumor; Neoplasm ; Meningioma ; Papillary meningioma ; Optic nerve sheath meningioma KEY WORDS :

I have reviewed 292 primary intraorbital meningiomas from the literature and found none to be papillary in type (23) . Moreover, I reviewed 122 primary papillary meningiomas from the literature (1-6,8-11,13,15-19, 21,25,26) . Two cases were excluded (12,20) because they were apparently included in the series of Ludw in et al (13) . There was no papillary meningioma of the sheath of the optic nerve among 122 papillary meningiomas . To my knowledge, this is the first reported instance of primary papillary meningioma of the sheath of the optic nerve with benign morphological, behavioral, and clinical features . Address reprint requests to : Samruay Shuangshoti, M .D., Department of Pathology, Chulalongkorn Hospital, Bangkok 10330, Thailand . Received July 29, 1992 ; accepted October 6, 1992 .

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1993 by Elsevier Science Publishing Co ., Inc .

Case Report A 54-year-old woman had blurred vision that gradually progressed in the left eye for 20 years . Ten years ago, proptosis developed on her left side . Six months prior to hospitalization, her left eye was blind and the exophthalmos was more advanced . Upon hospitalization, visual acuity was 20/20 in the right eye . In addition to amaurosis, the left eye showed limited upward gaze, injected conjunctiva, and an intact cornea . The round pupils, 5 mm across, were reactive to light. The lenses were clear. The disc of the left optic nerve was atrophic . A computed tomographic (CT) scan of the orbits and brain with and without contrast enhancement in the axial plane disclosed a soft tissue mass with a density of 31-69 Housfield units in the intraconal region of the left orbit . The lesion was posterior and superior to the globe and caused exophthalmos (Figure 1) . The orbital walls were intact . The patient underwent exenteration of the left eye . The periosteum was stripped from the rim and walls up to the apex of the orbit . The bulbar conjunctiva, all extraocular muscles, orbital fibroadipose tissue, optic nerve, and eyeball were removed . Because a part of the optic nerve between the posterior pole of the eyeball and the distal orifice of the optic canal had been replaced by a tumor, the eyeball, then, was separated from the latter, and the neoplasm was submitted for pathological study . The patient was discharged 10 days after hospitalization . She was last seen 2 years and 6 months after surgical intervention, and was well . There was no recurrence of the neoplasm .

Pathological Examination A formalin-fixed, well-circumscribed, encapsulated, and rubbery mass measured 2 x 2 x 4 cm . Sections revealed solid, gray, and whorled surfaces . The tissue was embedded in paraffin and stained with hematoxylin and eosin (H & E) . The peroxidase-antiperoxidase indirect immunohistochemical method was also performed on 0090-3019/931$6 .00



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Figure 1 . CT appearance of primary Papillary meningioma of optic nerve sheath . (A) Plain axial scan of the orbits showing the left intraconallesion (arrowhead) posterior to the globe causing proptosis . (B) Postcontrast axial scan demonstrates enhancement of the left intraconal lesion (arrowhead) .

paraffin-embedded tissue sections for localization of vimentin, cytokeratin, epithelial membrane antigen (EMA), S-100 protein, and glial fibrillary acidic protein (GFAP) . Microscopically, numerous neoplastic cells were disseminated beneath the thick fibrous capsule that represented the ducal sheath of the optic nerve and was focally invaded by them . The syncytial neoplastic cells having highly vesicular or even vacuolar nuclei were recognized as meningocytes . There were plentiful spindle-shaped tumor cells interpreted as fibroblasts and were often found in streams or intertwined bundles . These meningocytes and fibroblasts formed occasional

Figure 2 . Histopathological picture of meningioma of the optic nerve sheath demonstrates whorls of benign neoplastic cells (H & E, x 400) .

whorls (Figure 2) . Many trabeculae of the tumor cells were also present, and there were a few distinctive papillae (Figure 3) . The lesion contained a few foci of myxomatous changes . Nuclear mitotic figures and necrotic foci were not detected in many sections examined . Immunohistochemically, the tumor cells showed strong positivity to vimentin, cytokeratin, EMA, and S100 protein but were GFAP-negative . The pathological diagnosis was papillary meningioma (mixed meningocytic and fibroblastic meningioma associated with papillary formation) of the sheath of the left optic nerve .



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Shuangshoti

Figure 3. Papillary formation in meningioma of the optic nerve sheath . Note a distinc-

tive papilla to be formed by central core of vascular connective tissue covered by tumor cells (H & E, x 400) .

Discussion The presence of meningocytes and fibroblasts with cellular whorls and nuclear vacuoles established the pathological diagnosis of a meningioma (22-24) . The immunohistochemical positivity to vimentin, cytokeratin, EMA, and S-100 protein of the neoplastic cells is an additional diagnostic feature of meningioma (7,14,27) . The GFAP is generally known to be positive in gliomas, particularly in astrocytomas . This immunostain, however, was also reported to be positive in some papillary meningiomas (3,2 1) . Hence, it is not helpful in distinguishing a papillary meningioma of the sheath of the optic nerve from a glioma of the optic nerve . Nevertheless, the GFAP negativity is regarded herewith to differentiate an optic nerve glioma from the current lesion . Review of 123 reported primary papillary meningiomas, including the present tumor, revealed the recorded age in 52 cases, sex in 58 instances, and location in 45 examples- The two youngest patients were 4-month-old female infants (21) . The oldest was an 86-year-old man (21) . The average age was 30 years for females, 43 years for males, and 35 years for an overall average of both sexes . About one-fourth of patients were in the first two decades of their lives . The finding on the age suggests the tendency of the papillary meningiomas to occur in young patients . The female/male ratio was 3 : 2. Of 45 primary papillary meningiomas of known location, 36 were intracranial- They were situated as follows : 20 tumors on the cerebral convexity (2-4,8,9,13,16,17,20,21,24,26), eight parasagittal neoplasms (7,13,15,17), four supratentorial tumors at the

cranial base (2,6,13), one neoplasm at the midbrain (IS), two tentorial tumors (11,25), and two neoplasms in the posterior cranial fossa (9,13) . Four primary papillary meningiomas were intraspinal (1,13,21,25) . Five primary papillary meningiomas outside the central nervous system consisted of one tumor each (intraosseous meningioma) of the petrousapex (17), subcutaneous neoplasm of the glabella, angle of the jaw (21), and sheath of the optic nerve (current case) . The location of these tumors suggests that papillary meningiomas tend to occur on the convexity and in the parasagittal region of the brain . It has been controversial as to whether papillary formation expresses malignancy in meningiomas . Many authors regarded the papillary structures to represent morphological and behavioral malignant features in meningiomas (2,5,10,11,13,17,21) . They observed a high number of pleomorphic nuclei and mitotic figures, frequent foci of necrosis, infiltration of the surrounding brain, recurrence, and even metastasis in such papillary meningiomas . A few authors, on the other hand, considered papillary formation to be a pattern of arrangement of neoplastic cells without malignant indication in meningiomas if the aforementioned malignant features were absent (25) . 1 have no experience to support or refute these observations ; however, I do consider the present papillary meningioma of the optic nerve sheath to have been benign as it did not have any pathological malignant feature. Clinically, it existed within the patient's orbit for 20 years without destruction of the bony walls . Moreover, there was no recurrence of tumor after a follow-up study of 2 .5 years .



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The author is currently in receipt of support from the Faculty of Medicine of Chulalongkorn University-China Medical Board Scholar Development Fund (1991-1994), Bangkok, Thailand, and is thankful to Dr . Chaiyen Ratanavijarn, the attending ophthalmologist, in permitting him to report this patient .

References I . Allegranza A, Mariani C, Passeriani A, Grazioli L . Meningioma papillare maligno . Istocitopathologia 1980 ;2 :193-9 . 2 . Brignolio P, Favero M . Consideration on the malignancy of papillary meningioma: Clinicopathological study of eight cases . Zentralbi Neurochir 1989 ;45 :79-84 . . 3 Buska H . Non-glial specificities of immunocytochemistry for the glial fibrillary acidic protein (GFAP) : Triple expression of GFAP, vimentin and cyrokeratin in papillary meningioma and metastasizing renal carcinoma. Acta Neuropathol (Bert) 1986 ;72 :43-54 . 4 . Cushing H, Eisenhardt L. Meningiomas : Their classification, regional behaviour, life history, and surgical end results . Springfield, Ill : Charles C Thomas, 1938 ;692-719 . 5 . Deen HG Jr, Scheirhauer BW, Ebersold MJ . Clinical and pathological study of meningiomas of the first two decades of life . J Neurosurg 1982 ;56 :317-22 . 6 . Goin DW, Surgical management of petrous apex meningioma . Laryngoscope 1979 ;89 :204-13 . 7 . Holden J, Dohman CL, Churg A . Immunohistochemistry of meningioma including the angioblastic type. J Neuropathol Exp Neurol 1989 ;46 :50-6 . 8. Inoue H, Tamara M, Koizumi H, Nakamura M, Nakamura H, Ohye Ch. Clinical pathology of malignant meningiomas. Acta Neurochir (Wien) 1984 ;73 :179-91 . 9. Kasantikul V, Shuangshoti S. Papillary and glandular meningioinas : Report of two cases . J Med Assoc Thai 1985 ;68 :276-81 . 10. Kepes JJ, Goldware S, Leoni R. Meningioma with pseudoglandular pattern . J Neuropathol Exp Neurol 1983 ;42 :61-8 . 11 . Kepes JJ, MacGee BE, Vergara G, Sil R . Malignant meningioma with extensive pulmonary metastases : A case report . J Kansas Med Sot 1971 ;72 :312-6 . 12 . Ludwin SK, Conley FK . Malignant meningioma metastasizing through the cerebrospinal pathways . J Neurol Neurosurg Psychiatry 1975 ;38:136-42 .

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13 . Ludwin SK, Rubinstein LJ, Russell DS . Papillary meningioma: A malignant variant of meningioma. Cancer 1975 ;36 :1363-73 . 14 . Meis JM Ordonez NG, Bruner JM . Meningiomas : An immunohistochemical study of 50 cases . Arch Pathol Lab Med 1986 ;110 :934-7 . 15 . Miller AA, Ramsden F. Malignant meningioma with extracranial metastases and seeding of the subarachnoid space and the ventricles . Pathol Europ 1972 ;7 :167-75 . 16. Nakagawa H, Nakajima 5, Murasawa A, Miiyama K, Ishiguro S . Papillary meningioma arising from the confluens sinuum with multidirectional extensions through venous sinuses . Surg Neurol 1989 ;32 :219-24 . 17 . Pasquier B, Gasnier F, Pasquier D, Keddari E, Morens N, Couderc P. Papillary meningioma : Clinicopathologic study of seven cases and review of the literature . Cancer 1986 ;58 :299305 . 18 . Piatt JH Jr, Campbell GA, Oakes WJ . Papillary meningioma involving the oculomotor nerve in an infant : Case report . J Neurosurg 1986 ;64 :808-12 . 19 . Roytta M, Vallfors P, Svendsen P, Sourander P . Recurrent meningioma: A combined clinical, neuroradiological and neuropathological study . Acta Neurol Scand Suppl 1982 ;65(90) :105-6 . 20. Russell DS. Meningeal tumours: A review. J Clin Pathol 1950;3 :191-211 . 21 . Russell DS, Rubinstein I.J. Pathology of tumours of the nervous system, 5th ed . London : Edward Arnold, 1989(460) :181-2 . 22 . Shuangshoti S . Meningioma of the optic nerve . Br J Ophrhalmol 1973 ;57 :265-9 . 23 . Shuangshoti S . Primary meningiomas outside the central nervous system . In : Al-Mefty 0, ed . Meningiomas . New York : Raven Press, 1991 :107-28 . 24 . Shuangshoti S, Hongsaprabhas C, Netsky MG . Metastasizing meningioma, Cancer 1970 ;26 :832-41 . 25 . Stefanko SZ, Mackay WM. Papillary meningioma. Acta Neuroparhol (Berl) 1981 ;(Suppl):126-8 . 26 . Su C-F, Shih C -J, Tsou C-K er al . Malignant meningiomas : Clinical and pathological study of 10 cases . J Formosan Med Assoc 1986;85 :608-23 . 27 . Theaker JM, Garter KC, Esiri MM, Fleming KA . Epithelial membrane antigen and cytokeratin expression by meningiomas : An immunohistochemical study . J Clin Pathol 1986 ;39:435-9 .