Scwannoma intramedular: relato de caso

Botelho, Carlos H. A.; Kalil, Ricardo K.; Masini, Marcos

doi:10.1590/S0004-282X1996000300023

Resumos

A localização intramedular de schwannoma é rara, correspondendo a 0,3% dos tumores nesta topografia. Os autores relatam o caso de uma paciente leucoderma de 52 anos, que apresentou sintomas de compressão devido à presença de schwannoma intramedular localizado em nível de C4 a C6. Não foram encontrados sinais de neurofibromatose, enfermidade que tem sido associada ao desenvolvimento da lesão. Os exames de ressonância magnética nuclear e a biópsia transoperatórIa foram fatores decisivos no planejamento e na execução do tratamento, ao estabelecer as características, localização e diagnóstico da lesão. Sua boa delimitação e sua localização posterior facilitaram a exérese total. O exame histopatológico transoperatórIo permitiu a instituição de procedimento cirúrgico adequado. A célula de Schwann não é normalmente encontrada no sistema nervoso central e sua presença neste local tem sido objeto de várias teorias expostas no texto deste trabalho, que aborda também revisão de aspectos clínicos, diagnóstico por imagem, patologia, diagnóstico diferencial e tratamento dos schwannomas. E provável que, com os métodos propedêuticos atualmente disponíveis, venha a ser encontrado maior número destas lesões no futuro.

schwannoma; medula espinhal; abordagem diagnostica e terapêutica

The intramedullary localization of schwannomas is rare, corresponding to 0.3% of all intraspinal tumors. The authors report the case of a 52 year-old white female patient that presented with symptoms of spinal compression by the presence of an intramedullary schwannoma at the level C4-C6. There were no symptoms of neurofibromatosis, entity frequently related to the lesion. The magnetic resonance imaging examination and the per-operatory biopsy were decisive factors in planning and executing the treatment , by establishing the characteristics, location and diagnosis of the lesion. Its delimitation and posterior location have facilitated total surgical exeresis. The transoperatively histopathologic examination allowed adequate surgical procedure. The Schwann cell is not found normally in the central nervous system and its presence in this site has been subject of many theories exposed in this paper, which proposes comprehensive review of the clinical aspects, imaging diagnosis, pathology, differential diagnosis and treatment of schwannomas. It is probable that, with the advances verified in the available diagnostic methods, a greater number of these lesions may be diagnosed in the future.

schwannoma; spinal cord; diagnostic and therapeutic approaches

Scwannoma intramedular: relato de caso

Intramedullary schwannoma: case report

Carlos H. A. Botelho^I; Ricardo K. Kalil^I; Marcos Masini^II

^IRede SARAH de Hospitais do Aparelho Locomotor - Hospital SARAH - Brasília. Serviços de Patologia

^IIRede SARAH de Hospitais do Aparelho Locomotor - Hospital SARAH - Brasília. Neurocirurgia

RESUMO

A localização intramedular de schwannoma é rara, correspondendo a 0,3% dos tumores nesta topografia. Os autores relatam o caso de uma paciente leucoderma de 52 anos, que apresentou sintomas de compressão devido à presença de schwannoma intramedular localizado em nível de C4 a C6. Não foram encontrados sinais de neurofibromatose, enfermidade que tem sido associada ao desenvolvimento da lesão. Os exames de ressonância magnética nuclear e a biópsia transoperatórIa foram fatores decisivos no planejamento e na execução do tratamento, ao estabelecer as características, localização e diagnóstico da lesão. Sua boa delimitação e sua localização posterior facilitaram a exérese total. O exame histopatológico transoperatórIo permitiu a instituição de procedimento cirúrgico adequado. A célula de Schwann não é normalmente encontrada no sistema nervoso central e sua presença neste local tem sido objeto de várias teorias expostas no texto deste trabalho, que aborda também revisão de aspectos clínicos, diagnóstico por imagem, patologia, diagnóstico diferencial e tratamento dos schwannomas. E provável que, com os métodos propedêuticos atualmente disponíveis, venha a ser encontrado maior número destas lesões no futuro.

Palavras-chave: schwannoma, medula espinhal, abordagem diagnostica e terapêutica.

ABSTRACT

The intramedullary localization of schwannomas is rare, corresponding to 0.3% of all intraspinal tumors. The authors report the case of a 52 year-old white female patient that presented with symptoms of spinal compression by the presence of an intramedullary schwannoma at the level C4-C6. There were no symptoms of neurofibromatosis, entity frequently related to the lesion. The magnetic resonance imaging examination and the per-operatory biopsy were decisive factors in planning and executing the treatment , by establishing the characteristics, location and diagnosis of the lesion. Its delimitation and posterior location have facilitated total surgical exeresis. The transoperatively histopathologic examination allowed adequate surgical procedure. The Schwann cell is not found normally in the central nervous system and its presence in this site has been subject of many theories exposed in this paper, which proposes comprehensive review of the clinical aspects, imaging diagnosis, pathology, differential diagnosis and treatment of schwannomas. It is probable that, with the advances verified in the available diagnostic methods, a greater number of these lesions may be diagnosed in the future.

Key words: schwannoma, spinal cord, diagnostic and therapeutic approaches.

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Full text available only in PDF format.

Dr. Carlos Henrique de Aguiar Botelho - Hopsital SARAH Unidade Brasília - Avenida W3 Sul, Quadra 501, SMHS - 70330-150 Brasília, DF - Brasil. FAX 061 319-1564

1. Adelman LS, Aronson SM. Intramedullary nerve fiber and Schwann cell proliferation within the spinal cord (schwannosis). Neurology 1972;22:726-731.
2. Aryanpur J, Long DM. Schwannoma of the medulla oblongata: case report. J Neurosurg 1988,69:446-449.
3. Cantore G, Ciapetta P, Delfini R, Vagnozzi R, Nicoletti A. Intramedullary spinal neurilemomas: report of two cases. J Neurosurg 1982;57:143-147.
4. Casadei GP, Komori T, Scheithauer BW, Miller GM, Parisi JE, Kelia PJ. Intracranial parenchymal schwannoma: a clinicopathological and neuroimaging study of nine cases. J Neurosurg 1993:79:217-222.
5. Daras M, Koppel BS, Heise CW, Mazzeo MJ, Poon TP, Duffy KR. Multiple spinal intradural schwannomas in the absence of von Recklinghausen's disease. Spine 1993; 18:2556-2559.
6. Deruaz JP, Janzer RC, Costa J. Cellular schwannomas of the intracranial and intraspinal compartment: morphological and immunological characteristics compared with classical benign schwannomas. J Neuropathol Exper Neurol 1993;527:114-118.
7. Gibson AAM, Hendrick EB, Conen PE. Intracerebral schwannomas: report of a case. J Neurosurg 1966;24:552-557.
8. Gorman PH, Rigamonti D, Joslyn JN. Intramedullary and extramedullary schwannoma of the cervical spinal cord: case report. Surg Neurol 1989;32:459-462.
9. Hamilton MG, Mac Rae ME. Peripheral nerve sheath tumors: pathology and treatment. Contemp Neurosurg 1992; 14:1-6.
10. Henderson FC, Crockard HA, Stevens JM. Spinal cord oedema due to venous stasis. Neuroradiology 1993;35:312-315.
11. Herregodts P, Vloebergs M, Schmedding E, Goossers A, SladnikT, D'Haens J. Solitary dorsal intramedullary schwannoma: case report. J Neurosurg 1991 ;74:816-820.
12. Hu HP, Huang QL. Signal intensity correlation of MRI with pathological findings in spinal neurinomas. Neuroradiology 1992;34:98-102.
13. Iraci G, Peserico L, Salar G. Intraspinal neurinomas and schwannomas: a clinical survey of 172 cases. Internal Surg 1971;56:289-303.
14. Koeppen AH, Ordinario AT, Barron KD. Aberrant intramedullary peripheral nerve fibers. Arch Neurol 1968;18:567-573.
15. Lang EF, Bridge C. Intramedullary spinal cord tumors. Surg Clin North Am 1959;39:831-839.
16. Lesoin F, Delandsheer E, Krivosic 1, Clarisse J, Arnott G, Jomin M, Boudrez B.. Solitary intramedullary schwannomas. Surg Neurol 1983;19:51-56.
17. Masini M, Freire NG Neto. Procedimentos adicionais no tratamento de tumores raquianos: experiência com 100 casos. Arq Bras Neurocirurg 1988;7:9-28.
18. Mason TH, Keigher HA. Intramedullary spinal neurilemoma: case report. J Neurosurg 1968;29:414-416.
19. McCormick WF. Intramedullary spinal cord schwannoma: a unique case. Arch Pathol 1964;77:378-382.
20. Nagasawa S, OhtaT, KajimotoY, Aoki J, Onomura T, Miyagi X. Giant neurinoma occupying the holocervical and upper thoracic regions: case report. Surg Neurol 1994;42:157-159.
21. New P. Intracerebral schwannoma: case report. J Neurosurg 1972;36:795-797.
22. Pardatcher K, Iraci G, Capellotto P, Rigobello L, Pellone M, Fiore D. Multiple intramedullary neurinomas of the spinal cord: case report. J Neurosurg 1979;50:817-822.
23. Prakash B, Roy S, Tandon P. Schwannoma of the brain stem: case report. J Neurosurg 1980;53:121-123.
24. Riggs HE, Clary WV. A case of intramedullary sheath cell tumor of the spinal cord: consideration of vascular nerves as a source of origin. J Neuropathol Exper Neurol 1957; 16:332-336.
25. Ross DA, Edwards MSB, Wilson CB. Intramedullary neurilemomas of the spinal cord: report of two cases and review of the literature.Neurosurgery 1986;19:458-464.
26. Rout D, Pillai SM, Radhakrishnan VV. Cervical intramedullary schwannoma: case report. J Neurosurg 1983;58:962-964.
27. Scott M, Bentz R. Intramedullary neurilemoma (neurinoma) of the thoracic cord: a case report. J Neuropathol Exper Neurol 1962;21:194-200.
28. Shamji FM, Todd TR, Vallières E, Sachs HJ, Benoit BC. Central neurogenic tumors of the thoracic region. CIS 1992;35.
29. Souza AA, Vega MG, Dantas FR, Andrade MR. Neurinoma intramedular: relato de caso. Arq Bras Neurocirurg 1990;9:169-174.
30. Tabatabai A, Jungreis CA, Yonas H. Cervical schwannoma masquerading as a glioma: MR findings. J Comput Assist Tomogr 1990;14:489-490.
31. White W, Shiu MH, Rosenblum MK, Erlandson RA, Woodruff JM. Cervical schwannoma: a clinicopathologic study of 57 patients and 58 tumors. Cancer 1990;66:1266-1275.
32. Wood WG, Rothman LM, Nussbaum BE. Intramedullary neurilemoma of the cervical spinal cord: case report. J Neurosurg 1975;42:465-468.

Datas de Publicação

Publicação nesta coleção
10 Nov 2010
Data do Fascículo
Set 1996

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Adelman LS, Aronson SM. Intramedullary nerve fiber and Schwann cell proliferation within the spinal cord (schwannosis). Neurology 1972;22:726-731.

[2] 2. Aryanpur J, Long DM. Schwannoma of the medulla oblongata: case report. J Neurosurg 1988,69:446-449.

[3] 3. Cantore G, Ciapetta P, Delfini R, Vagnozzi R, Nicoletti A. Intramedullary spinal neurilemomas: report of two cases. J Neurosurg 1982;57:143-147.

[4] 4. Casadei GP, Komori T, Scheithauer BW, Miller GM, Parisi JE, Kelia PJ. Intracranial parenchymal schwannoma: a clinicopathological and neuroimaging study of nine cases. J Neurosurg 1993:79:217-222.

[5] 5. Daras M, Koppel BS, Heise CW, Mazzeo MJ, Poon TP, Duffy KR. Multiple spinal intradural schwannomas in the absence of von Recklinghausen's disease. Spine 1993; 18:2556-2559.

[6] 6. Deruaz JP, Janzer RC, Costa J. Cellular schwannomas of the intracranial and intraspinal compartment: morphological and immunological characteristics compared with classical benign schwannomas. J Neuropathol Exper Neurol 1993;527:114-118.

[7] 7. Gibson AAM, Hendrick EB, Conen PE. Intracerebral schwannomas: report of a case. J Neurosurg 1966;24:552-557.

[8] 8. Gorman PH, Rigamonti D, Joslyn JN. Intramedullary and extramedullary schwannoma of the cervical spinal cord: case report. Surg Neurol 1989;32:459-462.

[9] 9. Hamilton MG, Mac Rae ME. Peripheral nerve sheath tumors: pathology and treatment. Contemp Neurosurg 1992; 14:1-6.

[10] 10. Henderson FC, Crockard HA, Stevens JM. Spinal cord oedema due to venous stasis. Neuroradiology 1993;35:312-315.

[11] 11. Herregodts P, Vloebergs M, Schmedding E, Goossers A, SladnikT, D'Haens J. Solitary dorsal intramedullary schwannoma: case report. J Neurosurg 1991 ;74:816-820.

[12] 12. Hu HP, Huang QL. Signal intensity correlation of MRI with pathological findings in spinal neurinomas. Neuroradiology 1992;34:98-102.

[13] 13. Iraci G, Peserico L, Salar G. Intraspinal neurinomas and schwannomas: a clinical survey of 172 cases. Internal Surg 1971;56:289-303.

[14] 14. Koeppen AH, Ordinario AT, Barron KD. Aberrant intramedullary peripheral nerve fibers. Arch Neurol 1968;18:567-573.

[15] 15. Lang EF, Bridge C. Intramedullary spinal cord tumors. Surg Clin North Am 1959;39:831-839.

[16] 16. Lesoin F, Delandsheer E, Krivosic 1, Clarisse J, Arnott G, Jomin M, Boudrez B.. Solitary intramedullary schwannomas. Surg Neurol 1983;19:51-56.

[17] 17. Masini M, Freire NG Neto. Procedimentos adicionais no tratamento de tumores raquianos: experiência com 100 casos. Arq Bras Neurocirurg 1988;7:9-28.

[18] 18. Mason TH, Keigher HA. Intramedullary spinal neurilemoma: case report. J Neurosurg 1968;29:414-416.

[19] 19. McCormick WF. Intramedullary spinal cord schwannoma: a unique case. Arch Pathol 1964;77:378-382.

[20] 20. Nagasawa S, OhtaT, KajimotoY, Aoki J, Onomura T, Miyagi X. Giant neurinoma occupying the holocervical and upper thoracic regions: case report. Surg Neurol 1994;42:157-159.

[21] 21. New P. Intracerebral schwannoma: case report. J Neurosurg 1972;36:795-797.

[22] 22. Pardatcher K, Iraci G, Capellotto P, Rigobello L, Pellone M, Fiore D. Multiple intramedullary neurinomas of the spinal cord: case report. J Neurosurg 1979;50:817-822.

[23] 23. Prakash B, Roy S, Tandon P. Schwannoma of the brain stem: case report. J Neurosurg 1980;53:121-123.

[24] 24. Riggs HE, Clary WV. A case of intramedullary sheath cell tumor of the spinal cord: consideration of vascular nerves as a source of origin. J Neuropathol Exper Neurol 1957; 16:332-336.

[25] 25. Ross DA, Edwards MSB, Wilson CB. Intramedullary neurilemomas of the spinal cord: report of two cases and review of the literature.Neurosurgery 1986;19:458-464.

[26] 26. Rout D, Pillai SM, Radhakrishnan VV. Cervical intramedullary schwannoma: case report. J Neurosurg 1983;58:962-964.

[27] 27. Scott M, Bentz R. Intramedullary neurilemoma (neurinoma) of the thoracic cord: a case report. J Neuropathol Exper Neurol 1962;21:194-200.

[28] 28. Shamji FM, Todd TR, Vallières E, Sachs HJ, Benoit BC. Central neurogenic tumors of the thoracic region. CIS 1992;35.

[29] 29. Souza AA, Vega MG, Dantas FR, Andrade MR. Neurinoma intramedular: relato de caso. Arq Bras Neurocirurg 1990;9:169-174.

[30] 30. Tabatabai A, Jungreis CA, Yonas H. Cervical schwannoma masquerading as a glioma: MR findings. J Comput Assist Tomogr 1990;14:489-490.

[31] 31. White W, Shiu MH, Rosenblum MK, Erlandson RA, Woodruff JM. Cervical schwannoma: a clinicopathologic study of 57 patients and 58 tumors. Cancer 1990;66:1266-1275.

[32] 32. Wood WG, Rothman LM, Nussbaum BE. Intramedullary neurilemoma of the cervical spinal cord: case report. J Neurosurg 1975;42:465-468.