Sindrome de russel tumor diencefálico em uma criança

Motta, Luiz A. C. Roxo da; Garcia Filho, Osvaldo V.; Horta, Marcio P.; Lima, Benicio O.; Pratesi, Riccardo; Farage Filho, Miguel

doi:10.1590/S0004-282X1990000100016

Resumos

Apresenta-se o caso de criança do sexo masculino com síndrome de Russel devida a astrocitoma pilocítico localizado na região diencefálica. O diagnóstico foi realizado aos 16 meses de idade, mas sua sintomatologia se iniciou aos 4 meses, com emagrecimento progressivo. À internação apresentava-se com peso de 6150g e 74cm de estatura, emaciada, sem panículo adiposo, irritadica e com sinais de hipertensão intracraniana. Existia estrabismo convergente, nistagmo vertical do olho esquerdo e papiledema bilateral. Os reflexos tendinosos eram exaltados e tinha tetraparesia espástica. A avaliação endócrina demonstrou aumento basal do GH (23 ng/ml), do TSH (6,2 mUI/l) e da prolactina (26 ng/ml). Os dois primeiros hormônios não responderam ao teste agudo com o TRH, enquanto a prolactina teve resposta pobre. Foi submetida a radioterapia com acelerador linear (dose total de 4000 rads) e a cirurgia, na qual não se conseguiu extirpar todo o tumor, devido a seu tamanho. Nove meses após, a criança encontra-se bem, com apreciável ganho ponderai (2500g).

The case of a male child with Russel's syndrome due to a pilocytic astrocytoma located in the diencephalic region is presented. The diagnosis was made in the 16th month of age, but symptoms began in the 4th month of life, when he started losing weight By the time he was admitted weight was 6150g and he was 74cm tall, with an emaciated aspect, no panniculus adiposus, irritated, and with symptoms of intracranial hypertension. There was convergent strabismus, vertical nistagmus of the left eye and bilateral papilledema. Tendinous reflexes were exacerbated and he had spastic tetraparesis. The endocrine evaluation showed a basal raise of GH (23ng/ml), TSH (6.2mUI/l) and prolactin (26ng/ml). The first two hormones did not respond to the acute test with TRH, while prolactin had a poor response. He was submitted to radiotherapy with linear acceleration (total dose of 4000 rads) and surgery, during which the tumor could not be completely removed due to its large size. After 9 months, the child is doing well, with a considerable weight gain (2500g).

Luiz A. C. Roxo da Motta^I; Osvaldo V. Garcia Filho^II; Marcio P. Horta^III; Benicio O. Lima^III; Riccardo Pratesi^IV; Miguel Farage Filho^V

^IMédico do Núcleo de Neuroendocrinologia da Unidade de Neurocirurgia do HBDF e Serviço de Endocrinologia da Unb - Trabalho realizado na Unidade de Neurocirurgia do Hospital de Base do Distrito Federal (HBDF) e Serviço de Endocrinologia da Universidade de Brasília (UnB)

^IIResidente da Unidade de Neurocirurgia do HBDF - Trabalho realizado na Unidade de Neurocirurgia do Hospital de Base do Distrito Federal (HBDF) e Serviço de Endocrinologia da Universidade de Brasília (UnB)

^IIICirurgião do Setor de Neurocirurgia Pediátrica do HBDF - Trabalho realizado na Unidade de Neurocirurgia do Hospital de Base do Distrito Federal (HBDF) e Serviço de Endocrinologia da Universidade de Brasília (UnB)

^IVProfessor de Neurologia da UnB e médico da Unidade de Neurologia do HBDF - Trabalho realizado na Unidade de Neurocirurgia do Hospital de Base do Distrito Federal (HBDF) e Serviço de Endocrinologia da Universidade de Brasília (UnB)

^VChefe da Unidade de Neurocirurgia do HBDF - Trabalho realizado na Unidade de Neurocirurgia do Hospital de Base do Distrito Federal (HBDF) e Serviço de Endocrinologia da Universidade de Brasília (UnB)

RESUMO

Apresenta-se o caso de criança do sexo masculino com síndrome de Russel devida a astrocitoma pilocítico localizado na região diencefálica. O diagnóstico foi realizado aos 16 meses de idade, mas sua sintomatologia se iniciou aos 4 meses, com emagrecimento progressivo. À internação apresentava-se com peso de 6150g e 74cm de estatura, emaciada, sem panículo adiposo, irritadica e com sinais de hipertensão intracraniana. Existia estrabismo convergente, nistagmo vertical do olho esquerdo e papiledema bilateral. Os reflexos tendinosos eram exaltados e tinha tetraparesia espástica. A avaliação endócrina demonstrou aumento basal do GH (23 ng/ml), do TSH (6,2 mUI/l) e da prolactina (26 ng/ml). Os dois primeiros hormônios não responderam ao teste agudo com o TRH, enquanto a prolactina teve resposta pobre. Foi submetida a radioterapia com acelerador linear (dose total de 4000 rads) e a cirurgia, na qual não se conseguiu extirpar todo o tumor, devido a seu tamanho. Nove meses após, a criança encontra-se bem, com apreciável ganho ponderai (2500g).

SUMMARY

The case of a male child with Russel's syndrome due to a pilocytic astrocytoma located in the diencephalic region is presented. The diagnosis was made in the 16th month of age, but symptoms began in the 4th month of life, when he started losing weight By the time he was admitted weight was 6150g and he was 74cm tall, with an emaciated aspect, no panniculus adiposus, irritated, and with symptoms of intracranial hypertension. There was convergent strabismus, vertical nistagmus of the left eye and bilateral papilledema. Tendinous reflexes were exacerbated and he had spastic tetraparesis. The endocrine evaluation showed a basal raise of GH (23ng/ml), TSH (6.2mUI/l) and prolactin (26ng/ml). The first two hormones did not respond to the acute test with TRH, while prolactin had a poor response. He was submitted to radiotherapy with linear acceleration (total dose of 4000 rads) and surgery, during which the tumor could not be completely removed due to its large size. After 9 months, the child is doing well, with a considerable weight gain (2500g).

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Dr. Luiz A. C. Roxo da Motta - Núcleo de Neuroendocrinologia, Unidade de Neurocirurgia, Hospital de Base do Distrito Federal - SMHS Q 101 - 70333 Brasília DF - Brasil

1. Asa SL, Shceithaner BW, Bilbao RV, Horvalth E, Ryan N, Kovacs K, Randall RV, Laws ERJr, Singer W, Linfoot JA, Thorner MO, Vale W - A case for hypothalamic acromegaly: a clinicopathological study of six patients with hypothalamic gangliocytomas producing growth hormone-releasing factor. J Clin Endrocrinol Metab 53:796, 1984.
2. Burr IM, Slonim AE, Danish RK, Gedoth N, Butler IJ - Diencephalic syndrome revisited. J Pediatr 76:439, 1976.
3. Burrow GN, May PB. Spaulding SW, Donabedian RK - TRH and dopamine interactions affecting pituitary hormone secretion. J Clin Endrocrinol Metab 45:65, 1977.
4. Castro EM, Silva IN, Perpétuo FOL. - Síndrome de Russel: apresentação de um caso com desenvolvimento de puberdade precoce. Arq Neuro-Psiquiat (São Paulo) 45:72, 1987.
5. Hãger A, Thorell JI - Studies on growth hormone secretion in a patient with the diencephalic syndrome of emaciation. Acta Paediat Scand 62:231, 1973.
6. Hochman HI, Judge DM, Reichlin S - Precocious puberty and hypothalamic hamartoma. Pediatrics 67:236, 1981.
7. Joshi LR, Weintraub BD - Naturally occuring forms of thyrotropin with low bioactivity and altered carbohydrate content act as competitive antagonists to more bioactive forms. Endocrinology 113:2145, 1983.
8. Pimstone BL, Sobel J, Meyer E, Eale D - Secretion of growth hormone in the diencephalic syndrome of childhood. J Pedia.tr 76:886, 1970.
9. Reichilin S - Neuroendocrinology. In Wilson JD, Foster DW (eds): Williams Textbook of Endrocrinology. Ed 7. Saunders, Philadelphia, 1985, pg 1149.
10. Rossini AA, Cahill GFJr - Lipoatrophic diabetes. In Degroot LJ et al (eds): Endocrinology. Grune & Stratton, New York, 1981, pg 1093.
11. Salmon MA - Russel's diencephalic syndrome of early childhood. J Neurol Neurosurg Psychiat 35:196, 1972.
12. Spitz IM, Le Roith DL, Hirsh H, Carayon P, Pekonen F, Liel Y, Sobel R, Chorer Z, Weintraub B - Increased high-molecular-weight thyrotropin with impaired biologic activity in a euthyroid man. N Engl J Med 304:278, 1981.
13. Underwood LE, van Wyk JJ - Normal and aberrant growth. In Wilson JD, Foster DW (eds): Williams Textbook of Endrocrinology. Ed 7. Saunders, Philadelphia, 1985, pg 155.

Sindrome de russel tumor diencefálico em uma criança

Russel's syndrome: diencephalic tumor in a child

Datas de Publicação

Publicação nesta coleção
25 Maio 2011
Data do Fascículo
Mar 1990

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

[1] 1. Asa SL, Shceithaner BW, Bilbao RV, Horvalth E, Ryan N, Kovacs K, Randall RV, Laws ERJr, Singer W, Linfoot JA, Thorner MO, Vale W - A case for hypothalamic acromegaly: a clinicopathological study of six patients with hypothalamic gangliocytomas producing growth hormone-releasing factor. J Clin Endrocrinol Metab 53:796, 1984.

[2] 2. Burr IM, Slonim AE, Danish RK, Gedoth N, Butler IJ - Diencephalic syndrome revisited. J Pediatr 76:439, 1976.

[3] 3. Burrow GN, May PB. Spaulding SW, Donabedian RK - TRH and dopamine interactions affecting pituitary hormone secretion. J Clin Endrocrinol Metab 45:65, 1977.

[4] 4. Castro EM, Silva IN, Perpétuo FOL. - Síndrome de Russel: apresentação de um caso com desenvolvimento de puberdade precoce. Arq Neuro-Psiquiat (São Paulo) 45:72, 1987.

[5] 5. Hãger A, Thorell JI - Studies on growth hormone secretion in a patient with the diencephalic syndrome of emaciation. Acta Paediat Scand 62:231, 1973.

[6] 6. Hochman HI, Judge DM, Reichlin S - Precocious puberty and hypothalamic hamartoma. Pediatrics 67:236, 1981.

[7] 7. Joshi LR, Weintraub BD - Naturally occuring forms of thyrotropin with low bioactivity and altered carbohydrate content act as competitive antagonists to more bioactive forms. Endocrinology 113:2145, 1983.

[8] 8. Pimstone BL, Sobel J, Meyer E, Eale D - Secretion of growth hormone in the diencephalic syndrome of childhood. J Pedia.tr 76:886, 1970.

[9] 9. Reichilin S - Neuroendocrinology. In Wilson JD, Foster DW (eds): Williams Textbook of Endrocrinology. Ed 7. Saunders, Philadelphia, 1985, pg 1149.

[10] 10. Rossini AA, Cahill GFJr - Lipoatrophic diabetes. In Degroot LJ et al (eds): Endocrinology. Grune & Stratton, New York, 1981, pg 1093.

[11] 11. Salmon MA - Russel's diencephalic syndrome of early childhood. J Neurol Neurosurg Psychiat 35:196, 1972.

[12] 12. Spitz IM, Le Roith DL, Hirsh H, Carayon P, Pekonen F, Liel Y, Sobel R, Chorer Z, Weintraub B - Increased high-molecular-weight thyrotropin with impaired biologic activity in a euthyroid man. N Engl J Med 304:278, 1981.

[13] 13. Underwood LE, van Wyk JJ - Normal and aberrant growth. In Wilson JD, Foster DW (eds): Williams Textbook of Endrocrinology. Ed 7. Saunders, Philadelphia, 1985, pg 155.