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Isolated Schwannoma of the Olfactory Groove: A Case Report

Abstract

Introduction

Schwannoma of the olfactory groove is an extremely rare tumor that can share a differential diagnosis with meningioma or neuroblastoma.

Objectives

The authors present a case of giant schwannoma involving the anterior cranial fossa and ethmoid sinuses.

Case Report

The patient presented with a 30-month history of left nasal obstruction, anosmia, and sporadic ipsilateral bleeding. Computed tomography of the paranasal sinuses revealed expansive lesion on the left nasal cavity extending to nasopharynx up to ethmoid and sphenoid sinuses bilaterally with intraorbital and parasellar extension to the skull base. Magnetic resonance imaging scan confirmed the expansive tumor without dural penetration. Biopsy revealed no evidence of malignancy and probable neural cell. Bifrontal craniotomy was performed combined with lateral rhinotomy (Weber-Ferguson approach), and the lesion was totally removed. The tumor measured 8.0 4.3 3.7 cm and microscopically appeared as a schwannoma composed of interwoven bundles of elongated cells (Antoni A regions)mixed with less cellular regions (Antoni B). Immunohistochemical study stained intensively for vimentin and S-100.

Conclusion

Schwannomas of the olfactory groove are extremely rare, and the findings of origin of this tumor is still uncertain but recent studies point most probably to the meningeal branches of trigeminal nerve or anterior ethmoidal nerves.

paranasal sinuses; skull base; neurilemmoma; olfactory nerve; head and neck neoplasms; neoplasms; nerve tissue


Introduction

Schwannoma of the olfactory groove is extremely rare. So far only 35 cases have been reported in the literature when we searched the PubMed database. Schwannoma had already been described at the beginning of the 19th century, but Virchow was the first to recognize them for what they were. Such tumors account for ∼8% of all primary intracranial tumors.1Auer RN, Budny J, Drake CG, Ball MJ. Frontal lobe perivascular schwannoma. Case report. J Neurosurg 1982;56(1):154-157 The most common arises from the vestibular portion of the eighth nerve and, less commonly, the fifth, ninth, tenth, and twelfth cranial nerves.1Auer RN, Budny J, Drake CG, Ball MJ. Frontal lobe perivascular schwannoma. Case report. J Neurosurg 1982;56(1):154-1572Gibson AA, Hendrick EB, Conen PE. Case reports. Intracerebral schwannoma. Report of a case. J Neurosurg 1966;24(2):552-5573Ramamurthy B, Anguli V, Iyer CGS. A case of intramedullary neurinoma. J Neurosurg 1958;27:92-944Redekop G, Elisevich K, Gilbert J. Fourth ventricular schwannoma. Case report. J Neurosurg 1990;73(5):777-7815Amador AR, Santonja C, Del Pozo JM, Ortiz L. Olfactory schwannoma. Eur Radiol 2002;12(4):742-7446Sturm KW, Bonis G, Kosmaoglu V. [On a neurinoma of the cribriform lamina]. Zentralbl Neurochir 1968;29(4):217-222 They have been described as resembling subfrontal or olfactory groove schwannomas, which reflects their enigmatic origin.1Auer RN, Budny J, Drake CG, Ball MJ. Frontal lobe perivascular schwannoma. Case report. J Neurosurg 1982;56(1):154-1572Gibson AA, Hendrick EB, Conen PE. Case reports. Intracerebral schwannoma. Report of a case. J Neurosurg 1966;24(2):552-5573Ramamurthy B, Anguli V, Iyer CGS. A case of intramedullary neurinoma. J Neurosurg 1958;27:92-944Redekop G, Elisevich K, Gilbert J. Fourth ventricular schwannoma. Case report. J Neurosurg 1990;73(5):777-7815Amador AR, Santonja C, Del Pozo JM, Ortiz L. Olfactory schwannoma. Eur Radiol 2002;12(4):742-7446Sturm KW, Bonis G, Kosmaoglu V. [On a neurinoma of the cribriform lamina]. Zentralbl Neurochir 1968;29(4):217-2227Figueiredo EG, Soga Y, Amorim RL, Oliveira AM, Teixeira MJ. The puzzling olfactory groove schwannoma: a systematic review. Skull Base 2011;21(1):31-368Choi YS, Sung KS, Song YJ, Kim HD. Olfactory schwannoma-case report-. J Korean Neurosurg Soc 2009;45(2):103-1069Bezircioglu H, Sucu HK, Rezanko T, Minoglu M. Nasal-subfrontal giant schwannoma. Turk Neurosurg 2008;18(4):412-4141010 Sano H, Hayashi Y, Hasegawa M, Yamashita J. Subfrontal schwannoma without hyposmia-case report. Neurol Med Chir (Tokyo) 2004;44(11):591-5941111 Nagao S, Aoki T, Kondo S, Gi H, Matsunaga M, Fujita Y. [Subfrontal schwannoma: a case report]. No Shinkei Geka 1991;19(1):47-511212 Sato S, Toya S, Nakamura T, et al. [Subfrontal schwannoma: report of a case]. No Shinkei Geka 1985;13(8):883-8871313 Husain M, Mishra UK, Newton G, Husain N. Isolated olfactory groove neurilemmoma. Surg Neurol 1992;37(2):115-1171414 Sabel LH, Teepen JL. The enigmatic origin of olfactory schwannoma. Clin Neurol Neurosurg 1995;97(2):187-1911515 Adachi K, Yoshida K, Miwa T, Ikeda E, Kawase T. Olfactory schwannoma. Acta Neurochir (Wien) 2007;149(6):605-610, discussion 610-611 1616 Saberi H, Khashayar P. Olfactory groove schwannoma masquerading as an orbital mass. Neurosciences (Riyadh) 2008;13(1):73-761717 Frim DM, Ogilvy CS, Vonsattal JP, Chapman PH. Is intracerebral schwannoma a developmental tumor of children and young adults? Case report and review. Pediatr Neurosurg 1992;18 (4):190-1941818 Huang PP, Zagzag D, Benjamin V. Intracranial schwannoma presenting as a subfrontal tumor: case report. Neurosurgery 1997;40 (1):194-1971919 Yasuda M, Higuchi O, Takano S, Matsumura A. Olfactory ensheathing cell tumor: a case report. J Neurooncol 2006;76(2):111-1132020 Mirone G, Natale M, Scuotto A, Rotondo M. Solitary olfactory groove schwannoma. J Clin Neurosci 2009;16(3):454-4562121 Martínez-Soto L, Alfaro-Baca R, Torrecilla-Sardón MV, Fernández-Vallejo B, Ferreira-Muñóz R, De Diego T. [A new case of "olfactory schwannoma"; presentation and literature review]. Neurocirugia (Astur) 2009;20(3):294-2972222 Kanaan HA, Gardner PA, Yeaney G, et al. Expanded endoscopic endonasal resection of an olfactory schwannoma. J Neurosurg Pediatr 2008;2(4):261-2652323 Daglioglu E, Okay O, Dalgic A, Albayrak AL, Ergungor F. Cystic olfactory schwannoma of the anterior cranial base. Br J Neurosurg 2008;22(5):697-6992424 Yako K, Morita A, Ueki K, Kirino T. Subfrontal schwannoma. Acta Neurochir (Wien) 2005;147(6):655-657, discussion 657-658 2525 Komoribayashi N, Arai H, Kojo T, Obonai C, Wakabayashi J, Ogawa A. [Subfrontal schwannoma: case report]. No Shinkei Geka 2005;33(6):601-605

Case Report

The patient was a 39-year-old man with 30-month history of left nasal obstruction, anosmia, and sporadic ipsilateral bleeding. His physical examination showed a mass in the left nasal cavity extending to the nasopharynx. No café au lait spots or other signs of von Recklinghausen neurofibromatosis were observed.

Computed tomography of paranasal sinuses revealed expansive lesion on the left nasal cavity extending from the nasopharynx to ethmoid and sphenoid sinuses bilaterally with intraorbital and parasellar extension to the skull base. T2-weighted magnetic resonance imaging confirmed a heterogeneous hyperintense tumor occupying the anterior cranial fossa without dural penetration (Fig. 1). Biopsy by nasal endoscopy revealed no evidence of malignancy and probable neural cell. Cerebral angiography showed a large tumoral blush with pathologic vascularization of the displaced left maxillary artery and left ophthalmic artery without sufficient diameter for embolization.

Fig. 1
Coronal magnetic resonance imaging showing extensive mass growing down from cribriform plate.

The bilateral supraorbital approach was applied by making a coronal skin incision and retracting the scalp flap forward, and bilateral frontal craniotomy was performed with the John-Jane technique for excellent access to the floor of the anterior fossa and the superior orbits with less brain retraction. The orbital roof was partially removed and the dura was elevated on both sides of the crista galli. The tumor was seen arising from the base of the anterior cranial fossa and attached to the dura at level of the cribriform plate up to anterior clinoid process.

The lesion was well delineated, involving the anterior cranial fossa and parasellar region and extending down to the nasal cavity, nasopharynx, and ethmoid sinuses with intraorbital parasellar extension. The tumor was completely excised through the bifrontal craniotomy approach combined with lateral rhinotomy (Weber-Ferguson approach). The tumor measured 8.0 × 4.3 × 3.7 cm and was firm in consistency. Microscopically, the tumor appeared as a Schwannoma composed of interwoven bundles of elongated cells (Antoni A regions) mixed with less cellular regions (Antoni B). Immunohistochemical study stained intensively for vimentin and S-100 (Fig. 2). The resected area of the dura and tumor was repaired with part of the temporal bone and galea–periosteum aiming to minimize the possibility of fistula and infection. The patient remains free of disease with an excellent functional and cosmetic result. He is in good health with a residual sense of smell.

Fig. 2
Neopasm consisting of elongated spindle cells with poorly defined, palely eosinophil cytoplasm, sometime with nuclear palisading. Immunohistochemically, glial fibrillary acidic protein (GFAP) was negative and vimentin and S-100 protein stained intensively.

Discussion

Isolated schwannoma of the olfactory groove are rare tumors.1313 Husain M, Mishra UK, Newton G, Husain N. Isolated olfactory groove neurilemmoma. Surg Neurol 1992;37(2):115-1171414 Sabel LH, Teepen JL. The enigmatic origin of olfactory schwannoma. Clin Neurol Neurosurg 1995;97(2):187-1913535 Russell DS, Rubinstein LJ. Pathology of Tumors of the Nervous System. 3rd ed. London, UK: Edward Arnold; 1971:286 According to a review by Ulrich et al in 1978,3636 Ulrich J, Lévy A, Pfister C. Schwannoma of the olfactory groove. Case report and review of previous cases. Acta Neurochir (Wien) 1978;40(3-4):315-321 three of the previous cases had been reported in association with von Recklinghausen neurofibromatosis. Husain et al reported a case of a tumor arising from the base of the right anterior cranial fossa,1313 Husain M, Mishra UK, Newton G, Husain N. Isolated olfactory groove neurilemmoma. Surg Neurol 1992;37(2):115-117 and Sabel and Teepen referred to a case of tumor of the anterior cranial fossa where the olfactory bulb and nerve were not seen.1414 Sabel LH, Teepen JL. The enigmatic origin of olfactory schwannoma. Clin Neurol Neurosurg 1995;97(2):187-191

There are a few descriptions of intracerebral schwannomas.2Gibson AA, Hendrick EB, Conen PE. Case reports. Intracerebral schwannoma. Report of a case. J Neurosurg 1966;24(2):552-5573Ramamurthy B, Anguli V, Iyer CGS. A case of intramedullary neurinoma. J Neurosurg 1958;27:92-944Redekop G, Elisevich K, Gilbert J. Fourth ventricular schwannoma. Case report. J Neurosurg 1990;73(5):777-7811717 Frim DM, Ogilvy CS, Vonsattal JP, Chapman PH. Is intracerebral schwannoma a developmental tumor of children and young adults? Case report and review. Pediatr Neurosurg 1992;18 (4):190-1943737 Ben Rhouma T, Bouzakoura C, Boudaouara MA, Mhiri C, Hentati K. [Intracerebral schwannoma. Apropos of a case] . Neurochirurgie 1988;34(2):123-1273838 Ghatak NR, Norwood CW, Davis CH. Intracerebral schwannoma. Surg Neurol 1975;3(1):45-473939 Kasantikul V, Brown WJ, Cahan LD. Intracerebral neurilemmoma. J Neurol Neurosurg Psychiatry 1981;44(12):1110-11154040 Vassilouthis J, Richardson AE. Subfrontal schwannoma. Report of a case. Acta Neurochir (Wien) 1980;53(3-4):259-266 To date there are 35 cases of schwannoma of the anterior cranial fossa described in the literature.7Figueiredo EG, Soga Y, Amorim RL, Oliveira AM, Teixeira MJ. The puzzling olfactory groove schwannoma: a systematic review. Skull Base 2011;21(1):31-364141 Li YP, Jiang S, Zhou PZ, Ni YB. Solitary olfactory schwannoma without olfactory dysfunction: a new case report and literature review. Neurol Sci 2012;33(1):137-142

The origin of intracranial Schwannoma is intriguing. There are some hypotheses concerning their origin. Developmental theories hold that these lesions primarily arise from aberrant Schwann cells in the central nervous system.1414 Sabel LH, Teepen JL. The enigmatic origin of olfactory schwannoma. Clin Neurol Neurosurg 1995;97(2):187-1911515 Adachi K, Yoshida K, Miwa T, Ikeda E, Kawase T. Olfactory schwannoma. Acta Neurochir (Wien) 2007;149(6):605-610, discussion 610-611 1717 Frim DM, Ogilvy CS, Vonsattal JP, Chapman PH. Is intracerebral schwannoma a developmental tumor of children and young adults? Case report and review. Pediatr Neurosurg 1992;18 (4):190-1943333 Ahmad FU, Gupta A, Sharma MC, Shukla G, Mehta VS. The enigmatic origin of subfrontal schwannomas: report of a case without hyposmia. Acta Neurochir (Wien) 2006;148(6):671-672, discussion 672 Russell and Rubinstein advocate that mesenchymal pial cells may transform into ectodermal Schwann cells, explaining the occurrence of intraparenchymal schwannomas. Other theories suggest an origin from multipotent mesenchymal cells or displacement of neural crest cells forming the focus of Schwann cells within brain parenchyma (schwannosis).3535 Russell DS, Rubinstein LJ. Pathology of Tumors of the Nervous System. 3rd ed. London, UK: Edward Arnold; 1971:286

The developmental theories can explain the origin of intraparenchymal schwannomas,1414 Sabel LH, Teepen JL. The enigmatic origin of olfactory schwannoma. Clin Neurol Neurosurg 1995;97(2):187-1911515 Adachi K, Yoshida K, Miwa T, Ikeda E, Kawase T. Olfactory schwannoma. Acta Neurochir (Wien) 2007;149(6):605-610, discussion 610-611 1818 Huang PP, Zagzag D, Benjamin V. Intracranial schwannoma presenting as a subfrontal tumor: case report. Neurosurgery 1997;40 (1):194-1973333 Ahmad FU, Gupta A, Sharma MC, Shukla G, Mehta VS. The enigmatic origin of subfrontal schwannomas: report of a case without hyposmia. Acta Neurochir (Wien) 2006;148(6):671-672, discussion 672 but the subtype of schwannomas located in the anterior skull base intimately related to the olfactory groove seems to be an extra-axial tumor.1515 Adachi K, Yoshida K, Miwa T, Ikeda E, Kawase T. Olfactory schwannoma. Acta Neurochir (Wien) 2007;149(6):605-610, discussion 610-611 3131 Carron JD, Singh RV, Karakla DW, Silverberg M. Solitary schwannoma of the olfactory groove: case report and review of the literature. Skull Base 2002;12(3):163-1663333 Ahmad FU, Gupta A, Sharma MC, Shukla G, Mehta VS. The enigmatic origin of subfrontal schwannomas: report of a case without hyposmia. Acta Neurochir (Wien) 2006;148(6):671-672, discussion 672

Adachi et al reviewed the literature in 2007 about the origin of schwannoma from the fila olfactoria.1515 Adachi K, Yoshida K, Miwa T, Ikeda E, Kawase T. Olfactory schwannoma. Acta Neurochir (Wien) 2007;149(6):605-610, discussion 610-611 They reported that embryonic nerve, the plexus of dural vessels, and the fila olfactory that acquire Schwann cells have been considered as possible origins of these tumors.1515 Adachi K, Yoshida K, Miwa T, Ikeda E, Kawase T. Olfactory schwannoma. Acta Neurochir (Wien) 2007;149(6):605-610, discussion 610-611 1919 Yasuda M, Higuchi O, Takano S, Matsumura A. Olfactory ensheathing cell tumor: a case report. J Neurooncol 2006;76(2):111-113 However, others authors have pointed that the olfactory schwannomas originate from the olfactory bulb and nerve.8Choi YS, Sung KS, Song YJ, Kim HD. Olfactory schwannoma-case report-. J Korean Neurosurg Soc 2009;45(2):103-1061414 Sabel LH, Teepen JL. The enigmatic origin of olfactory schwannoma. Clin Neurol Neurosurg 1995;97(2):187-1911818 Huang PP, Zagzag D, Benjamin V. Intracranial schwannoma presenting as a subfrontal tumor: case report. Neurosurgery 1997;40 (1):194-1973333 Ahmad FU, Gupta A, Sharma MC, Shukla G, Mehta VS. The enigmatic origin of subfrontal schwannomas: report of a case without hyposmia. Acta Neurochir (Wien) 2006;148(6):671-672, discussion 672 A kind of cell that expresses phenotypic features of both astrocyte and Schwann cell is present.1919 Yasuda M, Higuchi O, Takano S, Matsumura A. Olfactory ensheathing cell tumor: a case report. J Neurooncol 2006;76(2):111-113

To assess the possibility of schwannomas originating from a real peripheral nerve, we need to know which nerve, present near the olfactory bulb and first cranial nerve, could be the origin of the tumor growth. The anterior ethmoidal nerve and the ramus meningeus of the trigeminal nerve are possibilities1313 Husain M, Mishra UK, Newton G, Husain N. Isolated olfactory groove neurilemmoma. Surg Neurol 1992;37(2):115-1171414 Sabel LH, Teepen JL. The enigmatic origin of olfactory schwannoma. Clin Neurol Neurosurg 1995;97(2):187-1914242 Viale ES, Pau A, Turtal S. Olfactory groove neurinomas. J Neurosurg Sci 1973;17:193-196 In most vertebrates, including man, a terminal nerve, which is part of an accessory olfactory system, is thought to be present. It is possible that this nerve is the source of an olfactory schwannoma.

The descriptions of the origin of this tumor are intriguing and enigmatic. Most descriptions point to a lesion intimately related to the anterior skull base, particularly the region of the olfactory groove.

Conclusion

Schwannomas represent ∼8% of all intracranial tumors. Schwannomas of the olfactory groove are extremely rare. The present case represents the 36th case described in the literature. The origin of this tumor is still uncertain but recent studies point most probably to the meningeal branches of trigeminal nerve or anterior ethmoidal nerves.

References

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    Ramamurthy B, Anguli V, Iyer CGS. A case of intramedullary neurinoma. J Neurosurg 1958;27:92-94
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    Redekop G, Elisevich K, Gilbert J. Fourth ventricular schwannoma. Case report. J Neurosurg 1990;73(5):777-781
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    Amador AR, Santonja C, Del Pozo JM, Ortiz L. Olfactory schwannoma. Eur Radiol 2002;12(4):742-744
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    Sturm KW, Bonis G, Kosmaoglu V. [On a neurinoma of the cribriform lamina]. Zentralbl Neurochir 1968;29(4):217-222
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    Figueiredo EG, Soga Y, Amorim RL, Oliveira AM, Teixeira MJ. The puzzling olfactory groove schwannoma: a systematic review. Skull Base 2011;21(1):31-36
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    Choi YS, Sung KS, Song YJ, Kim HD. Olfactory schwannoma-case report-. J Korean Neurosurg Soc 2009;45(2):103-106
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    Bezircioglu H, Sucu HK, Rezanko T, Minoglu M. Nasal-subfrontal giant schwannoma. Turk Neurosurg 2008;18(4):412-414
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    Sano H, Hayashi Y, Hasegawa M, Yamashita J. Subfrontal schwannoma without hyposmia-case report. Neurol Med Chir (Tokyo) 2004;44(11):591-594
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    Nagao S, Aoki T, Kondo S, Gi H, Matsunaga M, Fujita Y. [Subfrontal schwannoma: a case report]. No Shinkei Geka 1991;19(1):47-51
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    Husain M, Mishra UK, Newton G, Husain N. Isolated olfactory groove neurilemmoma. Surg Neurol 1992;37(2):115-117
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    Sabel LH, Teepen JL. The enigmatic origin of olfactory schwannoma. Clin Neurol Neurosurg 1995;97(2):187-191
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    Adachi K, Yoshida K, Miwa T, Ikeda E, Kawase T. Olfactory schwannoma. Acta Neurochir (Wien) 2007;149(6):605-610, discussion 610-611
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    Saberi H, Khashayar P. Olfactory groove schwannoma masquerading as an orbital mass. Neurosciences (Riyadh) 2008;13(1):73-76
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    Frim DM, Ogilvy CS, Vonsattal JP, Chapman PH. Is intracerebral schwannoma a developmental tumor of children and young adults? Case report and review. Pediatr Neurosurg 1992;18 (4):190-194
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    Huang PP, Zagzag D, Benjamin V. Intracranial schwannoma presenting as a subfrontal tumor: case report. Neurosurgery 1997;40 (1):194-197
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    Yasuda M, Higuchi O, Takano S, Matsumura A. Olfactory ensheathing cell tumor: a case report. J Neurooncol 2006;76(2):111-113
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    Mirone G, Natale M, Scuotto A, Rotondo M. Solitary olfactory groove schwannoma. J Clin Neurosci 2009;16(3):454-456
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    Martínez-Soto L, Alfaro-Baca R, Torrecilla-Sardón MV, Fernández-Vallejo B, Ferreira-Muñóz R, De Diego T. [A new case of "olfactory schwannoma"; presentation and literature review]. Neurocirugia (Astur) 2009;20(3):294-297
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    Yako K, Morita A, Ueki K, Kirino T. Subfrontal schwannoma. Acta Neurochir (Wien) 2005;147(6):655-657, discussion 657-658
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    Tsai YD, Lui CC, Eng HL, Liang CL, Chen HJ. Intracranial subfrontal schwannoma. Acta Neurochir (Wien) 2001;143(3):313-314
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    Ahmad FU, Gupta A, Sharma MC, Shukla G, Mehta VS. The enigmatic origin of subfrontal schwannomas: report of a case without hyposmia. Acta Neurochir (Wien) 2006;148(6):671-672, discussion 672
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    Bando K, Obayashi M, Tsuneharu F. [A case of subfrontal schwannoma]. No Shinkei Geka 1992;20(11):1189-1194
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    Russell DS, Rubinstein LJ. Pathology of Tumors of the Nervous System. 3rd ed. London, UK: Edward Arnold; 1971:286
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    Ulrich J, Lévy A, Pfister C. Schwannoma of the olfactory groove. Case report and review of previous cases. Acta Neurochir (Wien) 1978;40(3-4):315-321
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    Ben Rhouma T, Bouzakoura C, Boudaouara MA, Mhiri C, Hentati K. [Intracerebral schwannoma. Apropos of a case] . Neurochirurgie 1988;34(2):123-127
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    Ghatak NR, Norwood CW, Davis CH. Intracerebral schwannoma. Surg Neurol 1975;3(1):45-47
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    Kasantikul V, Brown WJ, Cahan LD. Intracerebral neurilemmoma. J Neurol Neurosurg Psychiatry 1981;44(12):1110-1115
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    Vassilouthis J, Richardson AE. Subfrontal schwannoma. Report of a case. Acta Neurochir (Wien) 1980;53(3-4):259-266
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    Li YP, Jiang S, Zhou PZ, Ni YB. Solitary olfactory schwannoma without olfactory dysfunction: a new case report and literature review. Neurol Sci 2012;33(1):137-142
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    Viale ES, Pau A, Turtal S. Olfactory groove neurinomas. J Neurosurg Sci 1973;17:193-196

Publication Dates

  • Publication in this collection
    Jan-Mar 2015

History

  • Received
    06 Feb 2013
  • Accepted
    01 July 2013
Fundação Otorrinolaringologia R. Teodoro Sampaio, 483, 05405-000 São Paulo/SP Brasil, Tel.: (55 11) 3068-9855, Fax: (55 11) 3079-6769 - São Paulo - SP - Brazil
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