Hemorragia maciça intratumoral em esclerose tuberosa estudo autóptico de um caso

Barbosa-Coutinho, Lígia M.; Lima, Eduardo Lahude; Gadret, Ricardo Olaechea; Ferreira, Nelson Pires

doi:10.1590/S0004-282X1991000400018

Resumos

O caso de um menino com esclerose tuberosa, apresentando o quadro clínico típico e que morreu com hemorragia intratumoral maciça, é estudado. A autópsia parcial de crânio revelou hemorragia cerebral maciça à esquerda, no interior da qual se identificou um tumor que, à microscopia, mostrou se tratar de astrocitoma subependimário de células gigantes. Este é o segundo caso, na literatura, no qual um paciente com esclerose tuberosa morre de hemorragia maciça intratumoral, com inundação ventricular.

A case of a 13-years-old boy with tuberous sclerosis manifested with the complete syndrome that died with a massive intratumoral hemorrhage is presented. The post mortem examination of the brain disclosed a massive left parenchymatous brain, hemorrhage with intraventricular component. In the hemorrhage, near the lateral ventricle wall, a subependymal giant cell astrocytoma was found. This is the second case in the literature of tuberous sclerosis which cause of death was an intratumoral massive hemorrhage.

CONTENTS CONTEÚDO

Hemorragia maciça intratumoral em esclerose tuberosa estudo autóptico de um caso

Massive intratumoral hemorrhage in tuberous sclerosis: autopsy study of a case

Lígia M. Barbosa-CoutinhoI; Eduardo Lahude LimaII; Ricardo Olaechea GadretIII; Nelson Pires FerreiraIV

IProfessora Adjunta do DP - Trabalho realizado no Departamento de Patologia (DP) da Fundação Faculdade Federal de Ciências Médicas de Porto Alegre (FFFCMPA)

IIMédico Residente do DP - Trabalho realizado no Departamento de Patologia (DP) da Fundação Faculdade Federal de Ciências Médicas de Porto Alegre (FFFCMPA)

IIIDoutorando da FFFCMPA - Trabalho realizado no Departamento de Patologia (DP) da Fundação Faculdade Federal de Ciências Médicas de Porto Alegre (FFFCMPA)

IVProfessor Adjunto de Neurocirurgia da FFFCMPA - Trabalho realizado no Departamento de Patologia (DP) da Fundação Faculdade Federal de Ciências Médicas de Porto Alegre (FFFCMPA)

RESUMO

O caso de um menino com esclerose tuberosa, apresentando o quadro clínico típico e que morreu com hemorragia intratumoral maciça, é estudado. A autópsia parcial de crânio revelou hemorragia cerebral maciça à esquerda, no interior da qual se identificou um tumor que, à microscopia, mostrou se tratar de astrocitoma subependimário de células gigantes. Este é o segundo caso, na literatura, no qual um paciente com esclerose tuberosa morre de hemorragia maciça intratumoral, com inundação ventricular.

SUMMARY

A case of a 13-years-old boy with tuberous sclerosis manifested with the complete syndrome that died with a massive intratumoral hemorrhage is presented. The post mortem examination of the brain disclosed a massive left parenchymatous brain, hemorrhage with intraventricular component. In the hemorrhage, near the lateral ventricle wall, a subependymal giant cell astrocytoma was found. This is the second case in the literature of tuberous sclerosis which cause of death was an intratumoral massive hemorrhage.

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Dra. Lígia Maria Barbosa Coutinho - Rua Carvalho Monteiro 414 - 90430 Porto Alegre RS - Brasil.

1. Askenasy HM, Behmoaram A. Subarachnoid haemorrhage in meningioma of the lateral ventricle. Neurology 1960, 10:484-489.
2. Balzer F, Menetrier P. Etude sur un cas d'adénomes sébacés de la face et du euir chevelu. Arch Physiol Norm Path 1885, 6:564-576.
3. Bourneville DM. Sclerose tubéreuse des circonvolutions cerebrales: idiotie et épilesie hémiplégique. Arch Neurol (Paris) 1880, 1:81-91.
4. Campbell AW. Cerebral sclerosis. Brain 1905, 28:382-396.
5. De Saussure RL Jr, Scheibert C, Hazoni LA. Astrocytoma grade III associated with profuse subarachnoidal bleeding as its first manifestation. J Neurosurg 1951, 8:236-239.
6. Donegani G, Grattarola FR, Wildi E. Tuberous sclerosis: Bourneville disease. In Vin-ken PJ, Bruyn GW. Handbook of Clinical Neurology. Amsterdam: North-Holland 1972, Vol 14 (The Phakomatoses) p 340-389.
7. Echols I>H, Rehfeldt FC. Profuse subarachnoid hemorrhage caused by cerebral glioma. J Neurosurg 1950, 7:280-282.
8. Eisenberg HM. Supratentorlal astrocytoma. In American Association of Neurological Surgeons (eds): Pediatric Neurosurgery, Surgery of Developing Nervous System. New York: Grune & Stratton, 1982, p 429-432.
9. Gruszkiewiez J, Doron Y, Gellei B, et al. Massive intracerebral bleeding due to supra-tentorial meningioma. Neurochirurgia 1975, 12:107-111.
10. Martuza RL. Neurofibromatosis and other phakomatoses. In Wilkins RH, Rengachary SS (eds): Neurosurgery. New York: McGraw-Hill, 1985, Vol 1, p 511-522.
11. McCoyd K, Barron KD, Cassidy RJ. Acoustic neurinoma presenting as subarachnoid hemorrhage: case report. J Neurosurg 1974, 41:391-393.
12. Morimoto K, Mogami H. Sequential CT study of subependymal giant-cell astrocytoma associated with tuberous sclerosis: case report. J Neurosurg 1986, 65:874-877.
13. Nardelli E, Benedictis G, La Stilla G, Nicolardi G. Tuberous sclerosis: a neuropatho-logical and immunohistochemical (PAP) study. Clin Neuropathol 1986, 5:261-266.
14. Osborne JP. Diagnosis of tuberous sclerosis. Arch Dis Child 1988, 63:1423-1425.
15. Pringle J J. Diagnosis of congenital adenoma sebaceum. Br J Derm 1890, 2:1-14.
16. von Recklinghausen F. Bin Herz von einem Neugeborenen, welches mehere theils nach aussen, theils nach der Hõhlen prominirende Tumoren (Myomen) trug. Monatschr Ge-burtskunde Frauenkrankenheiten 1862, 20:1-20.
17. Rovit RL, Fein JM. Pituitary apoplexy: a review and reappraisal. J Neurosurg 1972, 37:280-288.
18. Sampson JR, Yates JRW, Plrrit LA, Feury P, Winship I, Beighton P, Connor JM. Evidence for genetic heterogeneity in tuberous sclerosis. J Med Gen 1989, 26:511-516.
19. Sherlock EB. The Feeble-Minded. London: McMillan, 1911.
20. Waga S, Yamamoto Y, Kojima T, Sakakura M. Massive hemorrhage in tumor of tuberous sclerosis. Surg Neurol 1977, 8:99-101.
21. Yacubian EMT, Assumpção FB Jr, Duarte JC, da Cruz LM, Colarille LC, Marcucci M, Madruga MFL, Sprovieri MH, Funari SA, Lauandos TR, Ramos TCL. Esclerose tuberosa: estudo multidisciplinar de 15 casos. Arq Neuro-Psiquiat (São Paulo) 1983, 41:163-170.

Datas de Publicação

Publicação nesta coleção
22 Fev 2011
Data do Fascículo
Dez 1991

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

[1] 1. Askenasy HM, Behmoaram A. Subarachnoid haemorrhage in meningioma of the lateral ventricle. Neurology 1960, 10:484-489.

[2] 2. Balzer F, Menetrier P. Etude sur un cas d'adénomes sébacés de la face et du euir chevelu. Arch Physiol Norm Path 1885, 6:564-576.

[3] 3. Bourneville DM. Sclerose tubéreuse des circonvolutions cerebrales: idiotie et épilesie hémiplégique. Arch Neurol (Paris) 1880, 1:81-91.

[4] 4. Campbell AW. Cerebral sclerosis. Brain 1905, 28:382-396.

[5] 5. De Saussure RL Jr, Scheibert C, Hazoni LA. Astrocytoma grade III associated with profuse subarachnoidal bleeding as its first manifestation. J Neurosurg 1951, 8:236-239.

[6] 6. Donegani G, Grattarola FR, Wildi E. Tuberous sclerosis: Bourneville disease. In Vin-ken PJ, Bruyn GW. Handbook of Clinical Neurology. Amsterdam: North-Holland 1972, Vol 14 (The Phakomatoses) p 340-389.

[7] 7. Echols I>H, Rehfeldt FC. Profuse subarachnoid hemorrhage caused by cerebral glioma. J Neurosurg 1950, 7:280-282.

[8] 8. Eisenberg HM. Supratentorlal astrocytoma. In American Association of Neurological Surgeons (eds): Pediatric Neurosurgery, Surgery of Developing Nervous System. New York: Grune & Stratton, 1982, p 429-432.

[9] 9. Gruszkiewiez J, Doron Y, Gellei B, et al. Massive intracerebral bleeding due to supra-tentorial meningioma. Neurochirurgia 1975, 12:107-111.

[10] 10. Martuza RL. Neurofibromatosis and other phakomatoses. In Wilkins RH, Rengachary SS (eds): Neurosurgery. New York: McGraw-Hill, 1985, Vol 1, p 511-522.

[11] 11. McCoyd K, Barron KD, Cassidy RJ. Acoustic neurinoma presenting as subarachnoid hemorrhage: case report. J Neurosurg 1974, 41:391-393.

[12] 12. Morimoto K, Mogami H. Sequential CT study of subependymal giant-cell astrocytoma associated with tuberous sclerosis: case report. J Neurosurg 1986, 65:874-877.

[13] 13. Nardelli E, Benedictis G, La Stilla G, Nicolardi G. Tuberous sclerosis: a neuropatho-logical and immunohistochemical (PAP) study. Clin Neuropathol 1986, 5:261-266.

[14] 14. Osborne JP. Diagnosis of tuberous sclerosis. Arch Dis Child 1988, 63:1423-1425.

[15] 15. Pringle J J. Diagnosis of congenital adenoma sebaceum. Br J Derm 1890, 2:1-14.

[16] 16. von Recklinghausen F. Bin Herz von einem Neugeborenen, welches mehere theils nach aussen, theils nach der Hõhlen prominirende Tumoren (Myomen) trug. Monatschr Ge-burtskunde Frauenkrankenheiten 1862, 20:1-20.

[17] 17. Rovit RL, Fein JM. Pituitary apoplexy: a review and reappraisal. J Neurosurg 1972, 37:280-288.

[18] 18. Sampson JR, Yates JRW, Plrrit LA, Feury P, Winship I, Beighton P, Connor JM. Evidence for genetic heterogeneity in tuberous sclerosis. J Med Gen 1989, 26:511-516.

[19] 19. Sherlock EB. The Feeble-Minded. London: McMillan, 1911.

[20] 20. Waga S, Yamamoto Y, Kojima T, Sakakura M. Massive hemorrhage in tumor of tuberous sclerosis. Surg Neurol 1977, 8:99-101.

[21] 21. Yacubian EMT, Assumpção FB Jr, Duarte JC, da Cruz LM, Colarille LC, Marcucci M, Madruga MFL, Sprovieri MH, Funari SA, Lauandos TR, Ramos TCL. Esclerose tuberosa: estudo multidisciplinar de 15 casos. Arq Neuro-Psiquiat (São Paulo) 1983, 41:163-170.