RESUMO
The t(X;18)(p11.2;q11.2) (SYT/SSX1 or SSX2) is represented in more than 95% of synovial sarcoma. Even if recent data has implicated that the type of fusion gene (SYT/SSX1 or SYT/SSX2) can be of prognostic importance, the cellular and molecular mechanisms underlying the clinical behavior of synovial sarcoma are still poorly understood. To approach this issue, we investigated whether secondary genetic aberrations may influence the clinical outcome of synovial sarcoma. Clinical outcome with reference to comparative genomic hybridization (CGH) findings (losses or gains of genetic material) were analyzed for a uniquely large modern material of 69 synovial sarcomas. Thirty-five of 69 specimens showed DNA sequence copy number changes. The frequency of aberrations/tumor were higher (mean 4.7) for monophasic tumors than for biphasic tumors (mean 2.1). Gains of the whole or parts, including the long arm, of chromosome 8 were significantly overrepresented in large tumors (> 5 cm), suggesting that tumors with this genetic abnormality have an increased growth rate. No difference regarding metastasis-free or overall survival was seen between patients with or without tumors containing secondary copy number changes. No specific copy number change was linked to a significantly improved or impaired metastasis-free survival.
Assuntos
Aberrações Cromossômicas , Hibridização de Ácido Nucleico/métodos , Sarcoma Sinovial/genética , Neoplasias de Tecidos Moles/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , DNA/análise , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de TempoRESUMO
In a study based on formalin-fixed paraffin-embedded material from 86 patients with primary synovial sarcoma located in the extremities or on the trunk wall, the prognostic importance of MIB-1 index, p53-expession and tumour size was analysed. Multivariate analysis identified two metastatic risk factors: increasing tumour size and MIB-1 > 9%. The 5-year metastasis-free survival-rate for patients with tumour size < or = 5 cm + MIB-1 < 10% was 0.83 (95% confidence interval (CI) 0.64-0.92) compared to 0.31 (95% CI 0.11-0.53) in cases with tumour size > 5 cm + MIB-1 > or = 10%. Our study shows that metastatic disease in synovial sarcoma is closely related to MIB-1 index. Using our model based on tumour size and MIB-1 index, cases with good and poor prognosis can easily be discriminated. Therefore our model can be used to identify patients who should be considered for adjuvant chemotherapy.
Assuntos
Antígeno Ki-67/análise , Sarcoma Sinovial/mortalidade , Sarcoma Sinovial/patologia , Antígenos Nucleares , Biomarcadores/análise , Núcleo Celular/patologia , Intervalos de Confiança , Intervalo Livre de Doença , Humanos , Índice Mitótico , Análise Multivariada , Metástase Neoplásica , Proteínas Nucleares/análise , Prognóstico , Modelos de Riscos Proporcionais , Sistema de Registros , Estudos Retrospectivos , Países Escandinavos e Nórdicos/epidemiologia , Análise de Sobrevida , Proteína Supressora de Tumor p53/análiseAssuntos
Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/cirurgia , Amputação Cirúrgica , DNA de Neoplasias/análise , Análise Discriminante , Seguimentos , Humanos , Imuno-Histoquímica , Hibridização de Ácido Nucleico , Prognóstico , Sistema de Registros , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Fatores de Risco , Sarcoma Sinovial/genética , Sarcoma Sinovial/mortalidade , Países Escandinavos e Nórdicos/epidemiologia , Análise de Sobrevida , Translocação Genética , Resultado do TratamentoRESUMO
We analyzed treatment and outcome in 104 Scandinavian patients with synovial sarcoma in the extremities or trunk wall, diagnosed between 1986 and 1994. Only surgically treated patients without metastases at diagnosis were included. Median follow-up of survivors was 6 (3-11) years. 34 patients developed metastases. The overall 5- and 7-year survival rates were 0.76 (95% CI 0.66-0.83) and 0.69 (0.58-0.78), respectively. Large tumor size and amputation were significantly associated with impaired metastasis-free survival. Patients with local recurrence had a higher risk of metastases following the local event. Local excision with inadequate margin was associated with a higher risk of local recurrence.