Melanose neuro-cutanea: relato de um caso

Brandt, Reynaldo A.; Schwartzman, José Salomão; Naspitz, Charles

doi:10.1590/S0004-282X1978000200008

Resumos

É relatado o caso de um paciente do sexo masculino, de 3 meses de idade, com múltiplas lesões cutâneas, cuja biópsia revelou tratar-se de nevos, sem sinais de malignidade. Apresentou crises convulsivas refratárias ao tratamento medicamentoso e retardo de desenvolvimento psico-motor. Exames do líquido céfalorraqueano evidenciaram a presença de células neoplásicas, hipercitose, hiper-proteinorraquia e hipoglicorraquia. Pneumencefalografia revelou hidrocefalia discreta por bloqueio do espaço sub-aracnoideo. O estudo imunológico mostrou capacidade imunitária normal na fase inicial da doença e queda acentuada da mesma no seu estádio final. São feitos comentários baseados neste e em outros 43 casos descritos na literatura, destacando-se a importância dos exames laboratoriais na detecção de transformação maligna nas meninges em crianças portadoras de nevos benignos da pele.

The clinical, laboratorial and radiological features of a 3-month-old child with neurocutaneous melanosis are described. The patient was born with multiple disseminated benign cutaneous nevi, proven by skin-biopsy, and presented with drug-resistant seizures and psycho-motor retardation. Serial cerebrospinal fluid studies showed high protein and low glucose levels, with pleocytosis and malignant cells in the fluid. Pneumoencephalogram showed mild non-obstructive hydrocephalus. Immunological studies showed normal immunological activity at three months of age and very poor activity at one year. The patient died at 16 months of age, after the sudden onset of intracranial hypertension and meningeal signs. Immunotherapy and chemotherapy were suggested but not accepted by the family. Some comments are made based on this and other 43 cases described in the literature; the value of laboratory studies in detecting malignant transformation in the meninges in patients with benign skin nevi is stressed.

Reynaldo A. Brandt^I; José Salomão Schwartzman^II; Charles Naspitz^III

^INeurocirurgião

^IINeuropediatra

^IIIImunologista

RESUMO

É relatado o caso de um paciente do sexo masculino, de 3 meses de idade, com múltiplas lesões cutâneas, cuja biópsia revelou tratar-se de nevos, sem sinais de malignidade. Apresentou crises convulsivas refratárias ao tratamento medicamentoso e retardo de desenvolvimento psico-motor. Exames do líquido céfalorraqueano evidenciaram a presença de células neoplásicas, hipercitose, hiper-proteinorraquia e hipoglicorraquia. Pneumencefalografia revelou hidrocefalia discreta por bloqueio do espaço sub-aracnoideo. O estudo imunológico mostrou capacidade imunitária normal na fase inicial da doença e queda acentuada da mesma no seu estádio final. São feitos comentários baseados neste e em outros 43 casos descritos na literatura, destacando-se a importância dos exames laboratoriais na detecção de transformação maligna nas meninges em crianças portadoras de nevos benignos da pele.

SUMMARY

The clinical, laboratorial and radiological features of a 3-month-old child with neurocutaneous melanosis are described. The patient was born with multiple disseminated benign cutaneous nevi, proven by skin-biopsy, and presented with drug-resistant seizures and psycho-motor retardation. Serial cerebrospinal fluid studies showed high protein and low glucose levels, with pleocytosis and malignant cells in the fluid. Pneumoencephalogram showed mild non-obstructive hydrocephalus. Immunological studies showed normal immunological activity at three months of age and very poor activity at one year. The patient died at 16 months of age, after the sudden onset of intracranial hypertension and meningeal signs.

Immunotherapy and chemotherapy were suggested but not accepted by the family. Some comments are made based on this and other 43 cases described in the literature; the value of laboratory studies in detecting malignant transformation in the meninges in patients with benign skin nevi is stressed.

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Reynaldo A. Brandt Rua Murtinho Nobre 125 05502, São Paulo, SP Brasil.

1. BATTIN, J.; VITAL, C.; ALBERTY, J.; GUYONLT-DUPERAT, J. P.; LEGER, J. FONTAN, A. La mélanose neuro-cutanée. Arch. Franç. Péd. 25:277, 1968.
2. DOBSON, L. Prepubertal malignant melanomas: report of two cases. Amer. J. Surg. 89:1128, 1955.
3. FISHMAN, R. A. Cerebrospinal Fluid. In BAKER, A. B. Clinical Neurology. Vol. 1, Hoeber-Harper, New York, 1962, pp. 350-388.
4. FOX, H. Neurocutaneous melanosis, In VINKEN, P. J. & BRUYN, G. W. Handbook of Clinical Neurology, Vol. 14, North Holland, Amsterdam, 1972, pp. 414-428.
5. GIBSON, J. B.; BURROWS, D. & WEIR, W. P. Primary melanoma of the meniges. J. Pathol. 74:419, 1957.
6. HENDRIX, R. C. Juvenile melanomas, benign and malignant. A. M. A. Arch. Path. 58:636, 1954.
7. HJELT, L. & VILSKA, J. Malignant menigeal melanoma in a 3-year-old boy. Ann. Pedit. Fenn. 12:246, 1966.
8. HOFFMAN, H. J. & FREEMAN, A. Primary malignant leptomeningeal melanoma in association with giant hairy nevi. J. Neurosurg. 26:62, 1967.
9. JACOBSEN, H. H. & LESTER, H. A myelography manifestation of diffuse spinal leptomeningeal melanomatosis. Neuroradiology 1:30, 1970.
10. KAPLAN, A. M.; ITABASHI, H. H.; HANELIN, L. G. & LU, A. T. Neurocutaneous melanosis with malignant leptomeningeal melanoma: a case with metastases outside the nervous system. Arch. Neurol. (Chicago) 32:669. 1975.
11. KOCH, G. Genetic aspects of the phakomatose. In VINKEN, P. J. & BRUYN. G. W. Handobook of Clinical Neurology, Vol. 14, North-Holland, Amsterdam, 1972, pp. 488-561.
12. MC WHORTER, H. E. & SOOLNER, L. B. Pigmented nevi: juvenile melanomas and malignant melanomas in children, Cancer 7:564, 1954.
13. MORRIS, L. L. & DANTA, G. Malignant cerebral melanoma complicating giant pigmented naevus: a case report. J. Neurol. Neurosurg. Psychiat. 31:628, 1968.
14. NATHANSON, L.; HALL, T. C. & FARBER, S. Biological aspects of human malignant melanoma. Cancer 20:650, 1967.
15. REED, W. B.; BECKER, S. W.; BECKER JR., S. W. & NICKEL, W. R. Giant pigmented nevi, melanoma and leptomeningeal melanocytosis. Arch. Dermatol. 91: 100, 1965.
16. RUBINSTEIN, L. J. Tumors of the Central Nervous System. Armed Forces Institute of Pathology, Washington, 1972, pp. 309-311.
17. RUSSEL, J. L. & REYES, R. G. Giant pigmented nevi. JAMA 171:2083, 1959.
18. SALM, R. Primary malignant melanoma of the cerebellum. J. Pathol. 94:196, 1967.
19. SCHULTZ, R. C. Fatal malignant melanoma in children with giant nevi. Plast. Reconstr. Surg. 27:551, 1961.
20. SOBAJIMA, Y.; UCHIYAMA, T. & KUDO, H. An autopsy case of melanose neurocutanée (Touraine). Acta Path. Jap. 21:553, 1971.
21. SPITZ, S. Melanomas of childhood. Amer. J. Path. 3:591, 1948.
22. SWEET, L. K. & CONNERTY, H. V. Congenital melanoma; report of a case in which antenatal metastasis occured. Amer. J. Dis. Child. 62:1029, 1941.
23. TOLNAI, G.; CAMPBELL, J. S.; HILL, D. P.; PETERSON, E. W.; HUDSON, A. J. & LUNEY, F. W. Primary malignant melanomatosis of the leptomeninges. Arch. Neurol. (Chicago) 15:404, 1966.
24. TOURAINE, A. Les mélanoses neuro-cutanées. Ann. Derm. Syph. (Paris) 9:489, 1948.
25. TRUAX. K. F. & PAGE. H. G. Prepubertal malignant melanoma. Ann. Surg. 137:255, 1953.
26. VITAL, C.; BIOULAC, B.; GOT, M.; HENRY. P.; BEYLOT. J.; MORETTI, J. L. & LEBLANC, M. Étude clinique et anatomique d'un cas de mélanose neuro-cutanée. Bord. Med. 5:523, 1972.
27. WILCOX. J. C. Melanomatosis of the skin and central nervous system in infants. A congenital neurocutaneous syndrome. Amer. J. Dis. Child. 57:391. 1939.

Melanose neuro-cutanea: relato de um caso

Neurocutaneous melanosis: a case report

Datas de Publicação

Publicação nesta coleção
27 Ago 2012
Data do Fascículo
Jun 1978

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

[1] 1. BATTIN, J.; VITAL, C.; ALBERTY, J.; GUYONLT-DUPERAT, J. P.; LEGER, J. FONTAN, A. La mélanose neuro-cutanée. Arch. Franç. Péd. 25:277, 1968.

[2] 2. DOBSON, L. Prepubertal malignant melanomas: report of two cases. Amer. J. Surg. 89:1128, 1955.

[3] 3. FISHMAN, R. A. Cerebrospinal Fluid. In BAKER, A. B. Clinical Neurology. Vol. 1, Hoeber-Harper, New York, 1962, pp. 350-388.

[4] 4. FOX, H. Neurocutaneous melanosis, In VINKEN, P. J. & BRUYN, G. W. Handbook of Clinical Neurology, Vol. 14, North Holland, Amsterdam, 1972, pp. 414-428.

[5] 5. GIBSON, J. B.; BURROWS, D. & WEIR, W. P. Primary melanoma of the meniges. J. Pathol. 74:419, 1957.

[6] 6. HENDRIX, R. C. Juvenile melanomas, benign and malignant. A. M. A. Arch. Path. 58:636, 1954.

[7] 7. HJELT, L. & VILSKA, J. Malignant menigeal melanoma in a 3-year-old boy. Ann. Pedit. Fenn. 12:246, 1966.

[8] 8. HOFFMAN, H. J. & FREEMAN, A. Primary malignant leptomeningeal melanoma in association with giant hairy nevi. J. Neurosurg. 26:62, 1967.

[9] 9. JACOBSEN, H. H. & LESTER, H. A myelography manifestation of diffuse spinal leptomeningeal melanomatosis. Neuroradiology 1:30, 1970.

[10] 10. KAPLAN, A. M.; ITABASHI, H. H.; HANELIN, L. G. & LU, A. T. Neurocutaneous melanosis with malignant leptomeningeal melanoma: a case with metastases outside the nervous system. Arch. Neurol. (Chicago) 32:669. 1975.

[11] 11. KOCH, G. Genetic aspects of the phakomatose. In VINKEN, P. J. & BRUYN. G. W. Handobook of Clinical Neurology, Vol. 14, North-Holland, Amsterdam, 1972, pp. 488-561.

[12] 12. MC WHORTER, H. E. & SOOLNER, L. B. Pigmented nevi: juvenile melanomas and malignant melanomas in children, Cancer 7:564, 1954.

[13] 13. MORRIS, L. L. & DANTA, G. Malignant cerebral melanoma complicating giant pigmented naevus: a case report. J. Neurol. Neurosurg. Psychiat. 31:628, 1968.

[14] 14. NATHANSON, L.; HALL, T. C. & FARBER, S. Biological aspects of human malignant melanoma. Cancer 20:650, 1967.

[15] 15. REED, W. B.; BECKER, S. W.; BECKER JR., S. W. & NICKEL, W. R. Giant pigmented nevi, melanoma and leptomeningeal melanocytosis. Arch. Dermatol. 91: 100, 1965.

[16] 16. RUBINSTEIN, L. J. Tumors of the Central Nervous System. Armed Forces Institute of Pathology, Washington, 1972, pp. 309-311.

[17] 17. RUSSEL, J. L. & REYES, R. G. Giant pigmented nevi. JAMA 171:2083, 1959.

[18] 18. SALM, R. Primary malignant melanoma of the cerebellum. J. Pathol. 94:196, 1967.

[19] 19. SCHULTZ, R. C. Fatal malignant melanoma in children with giant nevi. Plast. Reconstr. Surg. 27:551, 1961.

[20] 20. SOBAJIMA, Y.; UCHIYAMA, T. & KUDO, H. An autopsy case of melanose neurocutanée (Touraine). Acta Path. Jap. 21:553, 1971.

[21] 21. SPITZ, S. Melanomas of childhood. Amer. J. Path. 3:591, 1948.

[22] 22. SWEET, L. K. & CONNERTY, H. V. Congenital melanoma; report of a case in which antenatal metastasis occured. Amer. J. Dis. Child. 62:1029, 1941.

[23] 23. TOLNAI, G.; CAMPBELL, J. S.; HILL, D. P.; PETERSON, E. W.; HUDSON, A. J. & LUNEY, F. W. Primary malignant melanomatosis of the leptomeninges. Arch. Neurol. (Chicago) 15:404, 1966.

[24] 24. TOURAINE, A. Les mélanoses neuro-cutanées. Ann. Derm. Syph. (Paris) 9:489, 1948.

[25] 25. TRUAX. K. F. & PAGE. H. G. Prepubertal malignant melanoma. Ann. Surg. 137:255, 1953.

[26] 26. VITAL, C.; BIOULAC, B.; GOT, M.; HENRY. P.; BEYLOT. J.; MORETTI, J. L. & LEBLANC, M. Étude clinique et anatomique d'un cas de mélanose neuro-cutanée. Bord. Med. 5:523, 1972.

[27] 27. WILCOX. J. C. Melanomatosis of the skin and central nervous system in infants. A congenital neurocutaneous syndrome. Amer. J. Dis. Child. 57:391. 1939.