Meningiomas múltiplos e neurofibromatose: relato de três casos

Oliveira, Mauro A.; Araujo, João F.M.; Balbo, Roque J.

doi:10.1590/S0004-282X1993000200017

Resumos

Meningiomas múltiplos «verdadeiros" geralmente não constituem entidade patológica específica. Em geral, associam-se a neurofibromatose. As clássicas lesões externas, descritas por von Recklinghausen, associadas à neurofibromatose, podem não estar presentes. Isto poderia ser possível devido à penetrância variável de aberrações cromossômicas ligadas ao cromossomo 22. Estudos moleculares desses tumores confirmam esta hipótese. Em nossa série de 108 pacientes com diagnóstico de meningioma intarcraniano, apenas três eram múltiplos. Apenas em um caso, estigmas externos de neurofibromatose foram encontrados. Os dois casos que não apresentavam qualquer estigma foram considerados como meningiomas múltiplos «verdadeiros". Na ausência de manifestações cutâneas habitualmente associadas à neurofibromatose, é extremamente difícil distinguir meningiomas múltiplos associados a neurofibromatose dos assim chamados meningiomas múltiplos verdadeiros. Em nossa opinião não há justificativa para se considerar meningiomas múltiplos como entidade patológica independente.

meningioma; meningiomas múltiplos; neurofibromatose

Multiple intracranial meningiomas (MIM) may be a specific pathological entity. In general these lesions are associated with neurofibromatosis. The classical clinical picture of neurofibromatosis, as described by von Recklinghausen, may not necessarily be associated with MIM. This possibility is a direct result of the variable penetrability of chromosomic aberrations connected with the chromosome 22. Molecular studies of these tumors confirmed this finding. In our series of 108 patients with intracranial meningiomas only three cases were multiple. In only one of them external stigmata of von Recklinghausen's disease were detected. In the absence of skin manifestations of neurofibromatosis in patients with MIM it is very difficult to diagnosis von Recklinghausen's disease, and the so called «true multiple meningiomas". The authors believe that there are no justificative findings to consider MIM as an independent pathological entity.

meningioma; multiple meningiomas; neurofibromatosis

Meningiomas múltiplos e neurofibromatose: relato de três casos

Multiple meningiomas and neurofibromatosis: report of three cases

Mauro A. Oliveira^I; João F.M. Araujo^I; Roque J. Balbo^II

^IProfessor Assistente e Neurocirurgião do HVC/HMMG. Departamento de Neuro-Psiquiatria da Faculdade de Ciências Médicas da Pontifícia Universidade Católica de Campinas (PUCCAMP), Departamento de Neurocirurgia do Hospital Vera Cruz (HVC) e Hospital Municipal «Dr. Mário Gatti», Campinas

^IIProfessor Adjunto e Diretor do Departamento de Neurocirurgia do HVC/HMMG. Departamento de Neuro-Psiquiatria da Faculdade de Ciências Médicas da Pontifícia Universidade Católica de Campinas (PUCCAMP), Departamento de Neurocirurgia do Hospital Vera Cruz (HVC) e Hospital Municipal «Dr. Mário Gatti», Campinas

RESUMO

Meningiomas múltiplos «verdadeiros" geralmente não constituem entidade patológica específica. Em geral, associam-se a neurofibromatose. As clássicas lesões externas, descritas por von Recklinghausen, associadas à neurofibromatose, podem não estar presentes. Isto poderia ser possível devido à penetrância variável de aberrações cromossômicas ligadas ao cromossomo 22. Estudos moleculares desses tumores confirmam esta hipótese. Em nossa série de 108 pacientes com diagnóstico de meningioma intarcraniano, apenas três eram múltiplos. Apenas em um caso, estigmas externos de neurofibromatose foram encontrados. Os dois casos que não apresentavam qualquer estigma foram considerados como meningiomas múltiplos «verdadeiros". Na ausência de manifestações cutâneas habitualmente associadas à neurofibromatose, é extremamente difícil distinguir meningiomas múltiplos associados a neurofibromatose dos assim chamados meningiomas múltiplos verdadeiros. Em nossa opinião não há justificativa para se considerar meningiomas múltiplos como entidade patológica independente.

Palavras-chave: meningioma, meningiomas múltiplos, neurofibromatose.

SUMMARY

Multiple intracranial meningiomas (MIM) may be a specific pathological entity. In general these lesions are associated with neurofibromatosis. The classical clinical picture of neurofibromatosis, as described by von Recklinghausen, may not necessarily be associated with MIM. This possibility is a direct result of the variable penetrability of chromosomic aberrations connected with the chromosome 22. Molecular studies of these tumors confirmed this finding. In our series of 108 patients with intracranial meningiomas only three cases were multiple. In only one of them external stigmata of von Recklinghausen's disease were detected. In the absence of skin manifestations of neurofibromatosis in patients with MIM it is very difficult to diagnosis von Recklinghausen's disease, and the so called «true multiple meningiomas". The authors believe that there are no justificative findings to consider MIM as an independent pathological entity.

Key words: meningioma, multiple meningiomas, neurofibromatosis.

Full text available only in PDF format.

Texto completo disponível apenas em PDF.

Aceite: 29-outubro-1992.

Dr. Mauro A. Oliveira Departamento de Neurocirurgia, Hospital Vera Cruz - Av. Andrade Neves 402 - 13013-160 Campinas SP - Brasil.

1. Anfimow J, Blumenaau L. Ein Fall multipler Geschwultes in der schadelhole. Neurol Zentralbl 1889, 8:585.
2. Battrsby RDE, Iriside JW, Malbty ED. Inherited multiple meningiomas: a clinical, pathological and cytogenetic study of an affected family. J Neurol Neurosurg Psychiatry 1986, 49:632-638.
3. Cushing H, Eisenhardt L. Meningiomas, their classification, regional behavior, life history and surgical results. Springfield Illinois: Thomas, 1938, p 115-132.
4. Davidoff LM, Martin J. Hereditary combined neurinomas and meningioma. J Neurosurg 1955, 12:375-384.
5. Delleman JW, De Jong GY, Blekeer GM. Meningiomas in five members of a family over two generations, in one member simultaneously with acustic neuromas. Neurology 1978, 28:567-570.
6. Deutsh EC. von Recklinghausen's neurofibromatosis with multiple intracranial tumors. Ear Throat J 1984, 63:141-148.
7. Ekong CEU, Paine KWE.. Rozdilsky B. Multiple meningiomas. Surg Neurol 1978, 9:181-183.
8. Etljamel MSM, Foy PM. Multiple meningiomas and their relation to neurofibromatosis. Surg Neurol 1989, 32:131-136.
9. Gaist G, Piazza G. Meningiomas in two numbers of the same family with no evidence of neurofibromatosis. J Neurosurg 1959, 16:110-113.
10. Horrax G. Meningiomas of the brain. Ach Neurol Psychiatr 1939, 41:140-157.
11. Joynt RJ, Perret GE. Meningiomas in a mother and daugther: cases without evidence of neurofibromatosis. Neurology 1985, 11:164-165.
12. Kunft HD, Protrowski W. Tiber gleichzeitiges Vorkommen primarer intrakranieller Ge-wachse verschiedener Keimblatter. Z Neurochir 1964, 24:189-198.
13. List CF. Multiple meningiomas: removal of four from region of the foramem magnum upper cervical region of the cord. Ach Neurol Psychiatr 1943, 50:335-341.
14. Lusins JO, Nakawa H. Multiple meningiomas evaluated by computed tomography. Neurosurgery 1981, 9:137-141.
15. Mashimayama S, Mori T, Seki H, Suzuki J. Multiple brain tumors with von Rekling-hausen's disease. Acta Neurochir (Wien) 1987, 84:29-35.
16. Menon MY. Multiple and familial meningiomas without evidence of neurofibromatosis. Neurosurgery 1980, 7:262-264.
17. Russell DS, Rubinstein LJ. Pathology of tumours of the nervous system. Ed 4, Baltimore: Willians and Wilkins, 1977, pp 50, 67, 70, 88, 311, 389.
18. Sheehy JP, Crockard A. Multiple meningiomas: a long term review. J Neurosurg 1983, 59:1-5.
19. Waga S, Miatsuda M, Handa H, Matsushima M, Ando K. Multiple meningiomas: report of four cases. J Neurosurg 1972, 37:348-351.
20. Winkelman NW. Post-operative seeding of the subarachnoid space and ventricles from a meningioma. J Neuropath Exp Neurol 1954, 13:260-266.
21. Wishart JH. Case of tumors in the skull, dura mater and brain. Edinburg Med Surg J 1922, 18:383-397.
22. Worster-Drought C, Dichson WEC, McMenemey WH. Multiple meningeal and perineural tumors with analogous changes in the gila and ependyma. Brain 1937, 60:85-117.
23. Wood NW, Whittle RJ, Kernohan JW. One hundred intracranial meningiomas found incidentally at necropsy. J Neuropath Exp Neurol 1957, 16:337-340.

Datas de Publicação

Publicação nesta coleção
19 Jan 2011
Data do Fascículo
Jun 1993

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

[1] 1. Anfimow J, Blumenaau L. Ein Fall multipler Geschwultes in der schadelhole. Neurol Zentralbl 1889, 8:585.

[2] 2. Battrsby RDE, Iriside JW, Malbty ED. Inherited multiple meningiomas: a clinical, pathological and cytogenetic study of an affected family. J Neurol Neurosurg Psychiatry 1986, 49:632-638.

[3] 3. Cushing H, Eisenhardt L. Meningiomas, their classification, regional behavior, life history and surgical results. Springfield Illinois: Thomas, 1938, p 115-132.

[4] 4. Davidoff LM, Martin J. Hereditary combined neurinomas and meningioma. J Neurosurg 1955, 12:375-384.

[5] 5. Delleman JW, De Jong GY, Blekeer GM. Meningiomas in five members of a family over two generations, in one member simultaneously with acustic neuromas. Neurology 1978, 28:567-570.

[6] 6. Deutsh EC. von Recklinghausen's neurofibromatosis with multiple intracranial tumors. Ear Throat J 1984, 63:141-148.

[7] 7. Ekong CEU, Paine KWE.. Rozdilsky B. Multiple meningiomas. Surg Neurol 1978, 9:181-183.

[8] 8. Etljamel MSM, Foy PM. Multiple meningiomas and their relation to neurofibromatosis. Surg Neurol 1989, 32:131-136.

[9] 9. Gaist G, Piazza G. Meningiomas in two numbers of the same family with no evidence of neurofibromatosis. J Neurosurg 1959, 16:110-113.

[10] 10. Horrax G. Meningiomas of the brain. Ach Neurol Psychiatr 1939, 41:140-157.

[11] 11. Joynt RJ, Perret GE. Meningiomas in a mother and daugther: cases without evidence of neurofibromatosis. Neurology 1985, 11:164-165.

[12] 12. Kunft HD, Protrowski W. Tiber gleichzeitiges Vorkommen primarer intrakranieller Ge-wachse verschiedener Keimblatter. Z Neurochir 1964, 24:189-198.

[13] 13. List CF. Multiple meningiomas: removal of four from region of the foramem magnum upper cervical region of the cord. Ach Neurol Psychiatr 1943, 50:335-341.

[14] 14. Lusins JO, Nakawa H. Multiple meningiomas evaluated by computed tomography. Neurosurgery 1981, 9:137-141.

[15] 15. Mashimayama S, Mori T, Seki H, Suzuki J. Multiple brain tumors with von Rekling-hausen's disease. Acta Neurochir (Wien) 1987, 84:29-35.

[16] 16. Menon MY. Multiple and familial meningiomas without evidence of neurofibromatosis. Neurosurgery 1980, 7:262-264.

[17] 17. Russell DS, Rubinstein LJ. Pathology of tumours of the nervous system. Ed 4, Baltimore: Willians and Wilkins, 1977, pp 50, 67, 70, 88, 311, 389.

[18] 18. Sheehy JP, Crockard A. Multiple meningiomas: a long term review. J Neurosurg 1983, 59:1-5.

[19] 19. Waga S, Miatsuda M, Handa H, Matsushima M, Ando K. Multiple meningiomas: report of four cases. J Neurosurg 1972, 37:348-351.

[20] 20. Winkelman NW. Post-operative seeding of the subarachnoid space and ventricles from a meningioma. J Neuropath Exp Neurol 1954, 13:260-266.

[21] 21. Wishart JH. Case of tumors in the skull, dura mater and brain. Edinburg Med Surg J 1922, 18:383-397.

[22] 22. Worster-Drought C, Dichson WEC, McMenemey WH. Multiple meningeal and perineural tumors with analogous changes in the gila and ependyma. Brain 1937, 60:85-117.

[23] 23. Wood NW, Whittle RJ, Kernohan JW. One hundred intracranial meningiomas found incidentally at necropsy. J Neuropath Exp Neurol 1957, 16:337-340.