Clinico-genetic aspects of a pediatric non-neurofibromatosis type 1 malignant triton tumor with loss of chromosome X
| Contribuinte(s) |
UNIVERSIDADE DE SÃO PAULO |
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| Data(s) |
12/09/2013
12/09/2013
2012
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| Resumo |
Malignant triton tumor (MTT) is an aggressive peripheral nerve sheath tumor with rhabdomyoblastic differentiation. Less than 100 cases have been described, being mostly male children with type 1 neurofibromatosis. We report a 6-year-old female with MTT and no diagnostic criteria for neurofibromatosis type 1. Cytogenetic analysis showed a 46,X,-X[4]/46,XX[16] karyotype. She underwent a transfemoral amputation and chemotherapy and is free of disease 15 months after diagnosis. The few cytogenetic studies of MTT described in the literature have been inconclusive. Further cytogenetic analyses are needed to understand the role of chromosome X monosomy in the pathogenesis of this rare tumor. Pediatr Blood Cancer 2012; 59: 13201323. (C) 2012 Wiley Periodicals, Inc. FAPESP (proc.) [2010/15717-0, 2010/16652-9] FAPESP (proc.) |
| Identificador |
PEDIATRIC BLOOD & CANCER, SAN FRANCISCO, v. 59, n. 7, supl. 1, Part 3, pp. 1320-1323, DEC, 2012 1545-5009 http://www.producao.usp.br/handle/BDPI/33283 10.1002/pbc.24197 |
| Idioma(s) |
eng |
| Publicador |
WILEY PERIODICALS, INC SAN FRANCISCO |
| Relação |
PEDIATRIC BLOOD & CANCER |
| Direitos |
closedAccess Copyright WILEY PERIODICALS, INC |
| Palavras-Chave | #CYTOGENETICS #MALIGNANT PERIPHERAL NERVE SHEATH TUMOR #MALIGNANT TRITON TUMOR #NEUROFIBROMATOSIS-1 #RHABDOMYOBLAST #NERVE SHEATH TUMOR #RHABDOMYOBLASTIC DIFFERENTIATION #GENE #ONCOLOGY #HEMATOLOGY #PEDIATRICS |
| Tipo |
article original article publishedVersion |
