Resumo
The clinical and pathological aspects of neurofibromatosis are summarized and an updated pathogeny of the tumor is made. The malignancy of the NFS senns to be linled to the NF - 1 gene mutations, with loss of the suppressor gene in the chromosone 17 pl. The surgical treatment is always useful because it lowers the pressure and relieves the patient.
Neurofibromatosis; Von Recklinghausen; Pelvic neurofibrosarcoma
Neurofibromatosis; Von Recklinghausen; Pelvic neurofibrosarcoma
RELATOS DE CASO
Neurofibrossarcoma associado a neurofibromatose
Neurofibrosarcoma associated to neurofibromatosis
Alcino Lázaro da Silva, TCBC. MGI; Venício do Amaral QuirinoII
IProfessor Titular de Cirurgia do Aparelho Digestivo
IIResidente de Cirurgia
Endereço para correspondência Endereço para correspondência: Dr. Alcino Lázaro da Silva Rua Guaratinga, 151 - Sion 30315-430 - Belo Horizonte - MG
ABSTRACT
The clinical and pathological aspects of neurofibromatosis are summarized and an updated pathogeny of the tumor is made. The malignancy of the NFS senns to be linled to the NF - 1 gene mutations, with loss of the suppressor gene in the chromosone 17 pl. The surgical treatment is always useful because it lowers the pressure and relieves the patient.
Key words: Neurofibromatosis; Von Recklinghausen; Pelvic neurofibrosarcoma.
Texto completo disponível apenas em PDF.
Full text available only in PDF format.
Recebido em 16/6/97
Aceito para publicação em 22/6/98
Trabalho do Departamento de Cirurgia da Faculdade de Medicina Federal de Minas Gerais.
- 1. Pitella JEH. Tumores intracranianos, intraraquianos e dos nervos periféricos. In: Bogliolo L - Bogliolo patologia - 4" ediçăo - Rio de Janeiro, Guanabara Koogan, 1987, cap. 26, pág. 780.
- 2. Menom AG, Anderson KM, Riccardi VM, et al. Chromosome 17p deletions and p53 gene mutations associated with the formation of malignant neurofibrosarcoma in Von Recklinghausen neurofibromatosis. Proc Natl Acad Sci USA 1990; 87(14); 5435-9.
- 3. Morioka N, Tshuchida T, Ishibashi Y, et al. A case of neurofibrosarcoma associated with neurofibromatosis-ganglioside analysis. Clin Exp Dermatol 1991;16(6);467-70.
- 4. Riccardi VM, Powel PP. Neurofibrosarcoma as a complication of Von Recklinghausen neurofibromatosis. Neurofibromatosis 1989; 2(3); 152-65.
- 5. Chanoki M, Ishii M, Fukai K, et al. Immunohistochemical localization of type I, III, IV, V and VI collagens and laminin in neurofibrorna and neurofibrosarcoma. Am J Dermatopathol 1991;13(4);365-73.
Datas de Publicação
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Publicação nesta coleção
23 Jul 2010 -
Data do Fascículo
Ago 1998
Histórico
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Aceito
22 Jun 1998 -
Recebido
16 Jun 1997