Assuntos
Neoplasias Encefálicas/tratamento farmacológico , Neoplasias Encefálicas/radioterapia , Glioma/tratamento farmacológico , Glioma/radioterapia , Adolescente , Neoplasias Encefálicas/mortalidade , Criança , Pré-Escolar , Terapia Combinada , Feminino , Seguimentos , Glioma/mortalidade , Humanos , Masculino , Prognóstico , Taxa de SobrevidaRESUMO
BACKGROUND: The role of postoperative radiotherapy and adjuvant chemotherapy in the treatment of synovial sarcoma remains to be determined. PROCEDURE: Twenty-five children were treated during a 23-year period with a multimodality approach. All of them had resection of the primary tumor (three amputations), followed by surgical retreatment in eight. Postoperative radiotherapy was delivered to 16 patients and adjuvant chemotherapy was given to 22. RESULTS: At the time of the report, 19 patients were alive and without evidence of disease. Six developed distant metastases (one associated with local recurrence); five of them died of their disease and one was alive in complete remission at 4 years from relapse. With a median follow-up of 9 years (range 2-23), the survival and the event-free survival at 5 years were 80% (SE 8.2) and 74% (SE 9.2), respectively. All relapsing patients had been classified as T2B. CONCLUSIONS: Multimodality treatment yielded satisfying survival results using limb-preserving surgery in most cases. Tumor size > 5 cm and invasiveness, which defined stage T2B, were the most important predictors of poor outcome. Evaluation of the role of adjuvant chemotherapy and radiotherapy awaits prospective studies, even if T2B patients, as well as children having nonradical surgery, seem worth managing by adjuvant treatments.
Assuntos
Sarcoma Sinovial/cirurgia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Braço/efeitos da radiação , Braço/cirurgia , Quimioterapia Adjuvante , Criança , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Perna (Membro)/efeitos da radiação , Perna (Membro)/cirurgia , Masculino , Invasividade Neoplásica , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Dosagem Radioterapêutica , Radioterapia Adjuvante , Reoperação , Estudos Retrospectivos , Sarcoma Sinovial/tratamento farmacológico , Sarcoma Sinovial/radioterapia , Sarcoma Sinovial/secundário , Taxa de Sobrevida , Resultado do TratamentoRESUMO
The Authors describe a case of CHARGE association (CA) with fibrocystic disease of the pancreas (FCP). The CA is characterized by seven mayor defects (colobomatous malformation, heart defect, atresia choanae, growth and mental deficiency, genital hypoplasia, ear anomalies and/or deafness). Four of this seven defects are necessary for the diagnosis. Other diseases and anomalies associated with CA are examined. The peculiarity of contemporaneous presence of FCP and the seriousness of the prognosis is pointed out.
