"Gliomatosis cerebri" simulating an acute diffuse encephalomyelitis: case report

Lancellotti, Carmen Lucia Penteado; Amary, Maria Fernanda Carriel; Barbastefano, Adriana M.; Tilbery, Charles Peter

doi:10.1590/S0004-282X1997000300023

Abstracts

Neuroradiologic, neuropathologic and immunohistochemical features are reported in a young man with a impairment of the central nervous system mimicking an acute diffuse encephalomyelitis. A white male, 17 years old, healthy till 4 months before, when developed a right hemiparesis and after 2 months a bilateral hemiparesis with a progressive impairment of several cranial nerves. Magnetic resonance imaging showed multiple lesions without a mass effect that suggested myelin loss. He remained unconscious for almost one month before dying of pneumonia. The neuropathologic examination showed a heavy brain (1505 g) with herniations and a large right midbrain. There were several soft and pink areas mainly at the right midbrain, left cerebellum and in the white matter of the left cerebral hemisphere. The histopathologic sections showed diffuse blastomatous proliferation without total replacement or destruction of the original tissue. The tumor cells had astrocytic, oligodendrocytic and spongioblastic phenotypes, some of them with a GFAP-positive reactivity. There were focal anaplastic changes. The diagnosis of "gliomatosis cerebri" was only possible by the autopsy.

Gliomatosis cerebri; neuroradiology; neuropathology; brain; tumour

São relatados os aspectos neurorradiológicos, neuropatológicos e imuno-histoquímicos em um paciente jovem com comprometimento do sistema nervoso central simulando encefalomielite aguda disseminada. Paciente masculino branco, com 17 anos de idade, hígido até há 4 meses, quando desenvolveu hemiparesia direita e, após 2 meses, hemiparesia bilateral com comprometimento progressivo de vários nervos cranianos. A imagem de ressonância magnética mostrou lesões múltiplas, sem efeito de massa, sugerindo perda da mielina. Permaneceu inconsciente durante quase 1 mês, com óbito decorrente de pneumonia. Na autópsia o encéfalo pesou 1505 g, com hérnias e tumefação do hemitronco direito. Havia várias áreas moles e róseas principalmente ao nível do hemitronco direito, cerebelo esquerdo e substância branca do hemisfério cerebral esquerdo. A histopatologia evidenciou proliferação blastomatosa difusa sem substituição ou destruição do tecido original. As células neoplásicas apresentavam fenotipos astrocíticos, oligodendrocíticos e espongioblásticos, algumas delas com reatividade positiva para GFAP. Havia transformação focai anaplásica. O diagnóstico de "gliomatosis cerebri" apenas foi possível com a autópsia.

gliomatosis cerebri; neurorradiologia; neuropatologia; cérebro; tumor

"Gliomatosis cerebri" simulating an acute diffuse encephalomyelitis: case report

"Gliomatosis cerebri" simulando encefalomielite disseminada aguda: relato de caso

Carmen Lucia Penteado Lancellotti^I; Maria Fernanda Carriel Amary^II; Adriana M. Barbastefano^III; Charles Peter Tilbery^IV

^IDepartamento de Ciências Patológicas e Disciplina de Neurologia da Faculdade de Ciências Médicas (FCM) da Santa Casa de São Paulo. Professor Adjunto de Patologia e Diretora do Departamento de Ciências Patológicas da FCM

^IIDepartamento de Ciências Patológicas e Disciplina de Neurologia da Faculdade de Ciências Médicas (FCM) da Santa Casa de São Paulo. Residente de 3º ano de Anatomia Patológica

^IIIDepartamento de Ciências Patológicas e Disciplina de Neurologia da Faculdade de Ciências Médicas (FCM) da Santa Casa de São Paulo. Auxiliar de Ensino de Neurologia

^IVDepartamento de Ciências Patológicas e Disciplina de Neurologia da Faculdade de Ciências Médicas (FCM) da Santa Casa de São Paulo. Professor Adjunto de Neurologia

ABSTRACT

Neuroradiologic, neuropathologic and immunohistochemical features are reported in a young man with a impairment of the central nervous system mimicking an acute diffuse encephalomyelitis. A white male, 17 years old, healthy till 4 months before, when developed a right hemiparesis and after 2 months a bilateral hemiparesis with a progressive impairment of several cranial nerves. Magnetic resonance imaging showed multiple lesions without a mass effect that suggested myelin loss. He remained unconscious for almost one month before dying of pneumonia. The neuropathologic examination showed a heavy brain (1505 g) with herniations and a large right midbrain. There were several soft and pink areas mainly at the right midbrain, left cerebellum and in the white matter of the left cerebral hemisphere. The histopathologic sections showed diffuse blastomatous proliferation without total replacement or destruction of the original tissue. The tumor cells had astrocytic, oligodendrocytic and spongioblastic phenotypes, some of them with a GFAP-positive reactivity. There were focal anaplastic changes. The diagnosis of "gliomatosis cerebri" was only possible by the autopsy.

Key words: Gliomatosis cerebri, neuroradiology, neuropathology, brain, tumour.

RESUMO

São relatados os aspectos neurorradiológicos, neuropatológicos e imuno-histoquímicos em um paciente jovem com comprometimento do sistema nervoso central simulando encefalomielite aguda disseminada. Paciente masculino branco, com 17 anos de idade, hígido até há 4 meses, quando desenvolveu hemiparesia direita e, após 2 meses, hemiparesia bilateral com comprometimento progressivo de vários nervos cranianos. A imagem de ressonância magnética mostrou lesões múltiplas, sem efeito de massa, sugerindo perda da mielina. Permaneceu inconsciente durante quase 1 mês, com óbito decorrente de pneumonia. Na autópsia o encéfalo pesou 1505 g, com hérnias e tumefação do hemitronco direito. Havia várias áreas moles e róseas principalmente ao nível do hemitronco direito, cerebelo esquerdo e substância branca do hemisfério cerebral esquerdo. A histopatologia evidenciou proliferação blastomatosa difusa sem substituição ou destruição do tecido original. As células neoplásicas apresentavam fenotipos astrocíticos, oligodendrocíticos e espongioblásticos, algumas delas com reatividade positiva para GFAP. Havia transformação focai anaplásica. O diagnóstico de "gliomatosis cerebri" apenas foi possível com a autópsia.

Palavras-chave: gliomatosis cerebri, neurorradiologia, neuropatologia, cérebro, tumor.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 1-abril-1997.

Dra. Carmen Lucia Penteado Lancellotti - Departamento de Anatomia Patológica, Santa Casa de São Paulo - Rua Dr. Cesario Morta Jr. 112 - 01277-900 São Paulo SP - Brasil.

1. Artigas J, Cervos-Navarro J, Iglesias JR, Erhhardt G. Gliomatosis cerebri: clinical and histological findings. Clin. Neuropathol, 1985;4:135-148.
2. Balakríshnan V, Hornabrook RW, Alexander WS. Gliomatosis cerebri: report of a case. Pathology, 1985;17:123-126.
3. Balko MG, Blisard KS, Samaha FJ. Oligodendroglial gliomatosis Cerebri. Hum Pathol, 1992;23:706-707.
4. Burger PC, Scheithauer BW. Atlas of tumor pathology: tumors of the central nervous system, 3^rd Series. Fascicle 10. Washington: Armed Forces Institute of Pathology, 1993:72-74.
5. Cervós-Navarro J, Artigas J, Aruffo C, Iglesias J. The fine structure of gliomatosis cerebri. Vlrchows Arch A 1987,411:93-98.
6. Couch JR, Weiss SA. Gliomatosis cerebri: report of four cases and review of the literature. Neurology, 1974;24:504-511.
7. Delisle MB, Andrieu P, Geraud G, Bouissou H. La gliomatose cérébrale: une observation anatomoclinique. Ann Pathol, 1989;9:214-218.
8. Dunn J, Kernohan JW. Gliomatosis cerebri. Arch. Pathol 1957;64:82-91.
9. Felsberg GJ, Silver SA, Brown MT.Tien RD. Radiologic-pathologic correlation: gliomatosis cerebri. AJNR, 1994; 15:1745-1753.
10. Galatioto S, Marafioti T, Cavallari V, Batolo D. Gliomatosis cerebri: clinical, neuropathological, immunohistochemical and morphometric studies. Zentralbl. Pathol 1993;139:261-267.
11. Hara A, Sakai N, Yamada H, Tanaka T, Mori H. Assessment of proliferative potential in gliomatosis cerebri. J Neurol 1991;238:80-82.
12. Jennings MT, Frenchman M, ShehabT, Johnson MD, Creasy J, Laporte K, Dettbarn WD. Gliomatosis cerebri presenting as intractable epilepsy during early childhood. J Child Neurol, 1995; 10:37-45.
13. Kleihues P, Burger PC, Scheithauer BW. Histological typing of tumors of the central nervous system, Ed 2. World Health Organization. International Histological Classification of Tumors. Berlin: Springer-Verlag, 1993:21-22.
14. Koslow SA, Claassen D, Hirsch WL, Jungreis CA. Gliomatosis cerebri: a case report with autopsy correlation. Neuroradiology 1992;34:331-333.
15. Miller RR, Lin F, Mallonee MM. Cytologic diagnosis of gliomatosis cerebri. Acta Cytol, 1981:25:37-39.
16. Mineura K, Sasajima T, Kowada M, Uesaka Y, Shishido F. Innovative Approach in the diagnosis of gliomatosis cerebri using carbon-11-methionine positron emission tomography. J Nucl Med 1991 ;32:726-728.
17. Nevin S. Gliomatosis cerebri. Brain 1938;61:170-191.
18. Ng HK. Diffuse gliomatosis of the central nervous system with histological features of microgliomatosis. Clin Neuropathol, 1988;7:266-270.
19. Priest JR, Wattweson J, Sung JH, Cox C. Gliomatosis cerebri following radiation and chemotherapy. J Neurosurg 1993;78:158-159.
20. Poser CM. Multiple sclerosis: diagnosis and treatment. Med. Principles Pract, 1992;3:1-16.
21. Poser CM. The epidemiology of multiple sclerosis: a general overview. Ann Neurol 1994;36:180-193.
22. Ross IB, Robitaille Y, Vlllemure JG, Tampieri D. Diagnosis and management of gliomatosis cerebri: recent trends. Surg Neurol, 1991;36:431-440.
23. Russell DS, Rubinstein U. Pathology of tumours of the nervous system, Ed 5. London: Arnold 1989:146-147.
24. Scherer HJ. The forms of growth in gliomas and their practical significance. Brain, 1940;63:1-35.
25. Schober R, Mai JK, Wechsler W. Gliomatosis cerebri: bioptical approach and neuropathological verification. Acta Neurochir (Wien), 1991,113:131-137.
26. Sheinker IM, Evans JP. Diffuse cerebral glioblastosis. J Neuropathol Exp Neurol 1943;2:178-189.
27. Shin YM, Chang KH, Han MH, Myung NH, Chi JG. Cha SH, Han MC. Gliomatosis cerebri: comparison of MT and CT features. AJR 1993;161:859-862.
28. Troost D, Kuiper H, Fleury P. Gliomatosis cerebri: report of a clinically diagnosed and histologically confirmed case. Clin Neurol Neurosurg 1987;89:43-47.
29. Wilson NW, Symon L, Lantos PL. Gliomatosis cerebri: report of a case presenting as a focal cerebral mass. J Neurol, 1987;234:445-447.
30. Yanaka K, Kamezaki T, Kobayashi E, Matsueda K, Yoshii Y, Nose T. MR imaging of diffuse glioma. AJNR, 1992;13:349-351.
31. Ziilch KJ. Brain tumors: their biology and pathology, Ed3. Berlin: Springer-Verlag 1986:341-342.

Publication Dates

Publication in this collection
18 Oct 2010
Date of issue
Sept 1997

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

[1] 1. Artigas J, Cervos-Navarro J, Iglesias JR, Erhhardt G. Gliomatosis cerebri: clinical and histological findings. Clin. Neuropathol, 1985;4:135-148.

[2] 2. Balakríshnan V, Hornabrook RW, Alexander WS. Gliomatosis cerebri: report of a case. Pathology, 1985;17:123-126.

[3] 3. Balko MG, Blisard KS, Samaha FJ. Oligodendroglial gliomatosis Cerebri. Hum Pathol, 1992;23:706-707.

[4] 4. Burger PC, Scheithauer BW. Atlas of tumor pathology: tumors of the central nervous system, 3^rd Series. Fascicle 10. Washington: Armed Forces Institute of Pathology, 1993:72-74.

[5] 5. Cervós-Navarro J, Artigas J, Aruffo C, Iglesias J. The fine structure of gliomatosis cerebri. Vlrchows Arch A 1987,411:93-98.

[6] 6. Couch JR, Weiss SA. Gliomatosis cerebri: report of four cases and review of the literature. Neurology, 1974;24:504-511.

[7] 7. Delisle MB, Andrieu P, Geraud G, Bouissou H. La gliomatose cérébrale: une observation anatomoclinique. Ann Pathol, 1989;9:214-218.

[8] 8. Dunn J, Kernohan JW. Gliomatosis cerebri. Arch. Pathol 1957;64:82-91.

[9] 9. Felsberg GJ, Silver SA, Brown MT.Tien RD. Radiologic-pathologic correlation: gliomatosis cerebri. AJNR, 1994; 15:1745-1753.

[10] 10. Galatioto S, Marafioti T, Cavallari V, Batolo D. Gliomatosis cerebri: clinical, neuropathological, immunohistochemical and morphometric studies. Zentralbl. Pathol 1993;139:261-267.

[11] 11. Hara A, Sakai N, Yamada H, Tanaka T, Mori H. Assessment of proliferative potential in gliomatosis cerebri. J Neurol 1991;238:80-82.

[12] 12. Jennings MT, Frenchman M, ShehabT, Johnson MD, Creasy J, Laporte K, Dettbarn WD. Gliomatosis cerebri presenting as intractable epilepsy during early childhood. J Child Neurol, 1995; 10:37-45.

[13] 13. Kleihues P, Burger PC, Scheithauer BW. Histological typing of tumors of the central nervous system, Ed 2. World Health Organization. International Histological Classification of Tumors. Berlin: Springer-Verlag, 1993:21-22.

[14] 14. Koslow SA, Claassen D, Hirsch WL, Jungreis CA. Gliomatosis cerebri: a case report with autopsy correlation. Neuroradiology 1992;34:331-333.

[15] 15. Miller RR, Lin F, Mallonee MM. Cytologic diagnosis of gliomatosis cerebri. Acta Cytol, 1981:25:37-39.

[16] 16. Mineura K, Sasajima T, Kowada M, Uesaka Y, Shishido F. Innovative Approach in the diagnosis of gliomatosis cerebri using carbon-11-methionine positron emission tomography. J Nucl Med 1991 ;32:726-728.

[17] 17. Nevin S. Gliomatosis cerebri. Brain 1938;61:170-191.

[18] 18. Ng HK. Diffuse gliomatosis of the central nervous system with histological features of microgliomatosis. Clin Neuropathol, 1988;7:266-270.

[19] 19. Priest JR, Wattweson J, Sung JH, Cox C. Gliomatosis cerebri following radiation and chemotherapy. J Neurosurg 1993;78:158-159.

[20] 20. Poser CM. Multiple sclerosis: diagnosis and treatment. Med. Principles Pract, 1992;3:1-16.

[21] 21. Poser CM. The epidemiology of multiple sclerosis: a general overview. Ann Neurol 1994;36:180-193.

[22] 22. Ross IB, Robitaille Y, Vlllemure JG, Tampieri D. Diagnosis and management of gliomatosis cerebri: recent trends. Surg Neurol, 1991;36:431-440.

[23] 23. Russell DS, Rubinstein U. Pathology of tumours of the nervous system, Ed 5. London: Arnold 1989:146-147.

[24] 24. Scherer HJ. The forms of growth in gliomas and their practical significance. Brain, 1940;63:1-35.

[25] 25. Schober R, Mai JK, Wechsler W. Gliomatosis cerebri: bioptical approach and neuropathological verification. Acta Neurochir (Wien), 1991,113:131-137.

[26] 26. Sheinker IM, Evans JP. Diffuse cerebral glioblastosis. J Neuropathol Exp Neurol 1943;2:178-189.

[27] 27. Shin YM, Chang KH, Han MH, Myung NH, Chi JG. Cha SH, Han MC. Gliomatosis cerebri: comparison of MT and CT features. AJR 1993;161:859-862.

[28] 28. Troost D, Kuiper H, Fleury P. Gliomatosis cerebri: report of a clinically diagnosed and histologically confirmed case. Clin Neurol Neurosurg 1987;89:43-47.

[29] 29. Wilson NW, Symon L, Lantos PL. Gliomatosis cerebri: report of a case presenting as a focal cerebral mass. J Neurol, 1987;234:445-447.

[30] 30. Yanaka K, Kamezaki T, Kobayashi E, Matsueda K, Yoshii Y, Nose T. MR imaging of diffuse glioma. AJNR, 1992;13:349-351.

[31] 31. Ziilch KJ. Brain tumors: their biology and pathology, Ed3. Berlin: Springer-Verlag 1986:341-342.