Clinical signs of neurofibromatosis impact on the outcome of malignant peripheral nerve sheath tumors.
Oncology
; 86(2): 122-6, 2014.
Article
em En
| MEDLINE
| ID: mdl-24480823
ABSTRACT
OBJECTIVE:
Malignant peripheral nerve sheath tumors (MPNST) are a rare subtype of sarcoma, with a poor outcome. MPNST are regarded as being sporadic or associated with neurofibromatosis type 1 (NF1). Few comparative overall-survival (OS) data in these 2 subsets of MPNST patients exist. The aim of this retrospective study was to assess OS in sporadic and NF1-associated MPNST patients.METHODS:
Fourteen consecutive patients with initial localized as well as initial metastatic MPNST were diagnosed and treated in our department. Patients with sporadic MPNST were assigned to group A and those with NF1-associated MPNST to group B.RESULTS:
Eight versus 6 patients were allocated to groups A and B. Primary tumors were located on the extremities in all but 1 patient. Two patients in group A and 4 patients in group B experienced a relapse. Four patients died in each of the 2 groups. Median follow-up was 66.2 and 57.2 months in group A and group B, respectively. Median OS in group A was 46.9 months versus 12.7 months in group B.CONCLUSIONS:
In this small, single-center study, sporadic-MPNST patients had a longer median OS than those with NF1-associated MPNST.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Protocolos de Quimioterapia Combinada Antineoplásica
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Neurofibromatose 1
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Neoplasias de Bainha Neural
Tipo de estudo:
Diagnostic_studies
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Observational_studies
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Risk_factors_studies
Limite:
Adult
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Aged
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Aged80
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Oncology
Ano de publicação:
2014
Tipo de documento:
Article
País de afiliação:
Áustria