Assuntos
Neoplasias Bucais/patologia , Neoplasias Nasais/patologia , Rabdomiossarcoma Alveolar/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Irradiação Craniana , Ciclofosfamida/administração & dosagem , Cistos/diagnóstico , Dactinomicina/administração & dosagem , Diagnóstico Diferencial , Feminino , Humanos , Mucosa Bucal/patologia , Neoplasias Bucais/diagnóstico por imagem , Neoplasias Bucais/tratamento farmacológico , Neoplasias Bucais/radioterapia , Mucosa Nasal/patologia , Neoplasias Nasais/diagnóstico por imagem , Neoplasias Nasais/tratamento farmacológico , Neoplasias Nasais/radioterapia , Rabdomiossarcoma Alveolar/diagnóstico por imagem , Rabdomiossarcoma Alveolar/tratamento farmacológico , Rabdomiossarcoma Alveolar/radioterapia , Ultrassonografia , Vincristina/administração & dosagemRESUMO
BACKGROUND: About 250-300 children with newly diagnosed cancer are treated each year at the Unidad Nacional de Oncologia Pediatrica in Guatemala City; less than 5% of them have soft tissue sarcomas (STS). The aim of the article was to evaluate whether the therapeutic standards achieved in STS in developed countries could be reproduced in a low-income country. PATIENTS AND METHODS: We reviewed the clinical data, treatment and outcome of 80 patients, 47 cases of rhabdomyosarcoma (RMS) and 33 of non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), treated between January 2000 and October 2007. RESULTS: Most of the RMS patients had advanced disease at diagnosis (87% groups III-IV). Their 3-year event-free survival rate (EFS) was 26.4% if abandoning the treatment was considered as an event, or 32.4% if it was censored (14 patients abandoned the treatment), and the 3-year overall survival rate (OS) was 43.5%. Local progression/relapse was the main cause of treatment failure. Among the patients with NRSTS, the EFS at 3 years was 36.4% (when abandoning the treatment was considered as an event) or 43.3% (when it was censored), and the OS was 44.2%. Outcome was satisfactory for synovial sarcoma patients, those with tumors < or =5 cm, and those with localized disease. CONCLUSIONS: Overall results were unsatisfactory compared to results reported from developed countries. Late diagnosis and the consequently high proportion of cases of advanced disease at diagnosis, the large number of patients failing to complete the treatment, and the poor quality of local control (in RMS) adversely influence outcome.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Países em Desenvolvimento , Rabdomiossarcoma Alveolar/tratamento farmacológico , Rabdomiossarcoma Embrionário/tratamento farmacológico , Neoplasias de Tecidos Moles/tratamento farmacológico , Adolescente , Quimioterapia Adjuvante , Criança , Pré-Escolar , Terapia Combinada , Feminino , Guatemala , Humanos , Lactente , Masculino , Prognóstico , Rabdomiossarcoma Alveolar/radioterapia , Rabdomiossarcoma Embrionário/radioterapia , Neoplasias de Tecidos Moles/radioterapia , Taxa de Sobrevida , Resultado do TratamentoRESUMO
PURPOSE: The authors present here 5 cases of continent urinary diversion in rhabdomyosarcoma applying a recently described technique for the Mitrofanoff Principle devised by the authors. METHODS: Two previously irradiated rhabdomyosarcoma patients presenting with residual bladder disease and massive sensitive urinary urgency underwent a transverse colonic reservoir with catheterizable stoma. Two other patients presenting with a Bricker conduit underwent conversion into an ileal reservoir. One patient underwent reconstruction after a cystectomy. RESULTS: All patients were continent and able to perform continent intermittent catheterization. CONCLUSIONS: The technique proved to be feasible for this group of patients. The authors believe that because of its simplicity, it should be an option of continent urinary diversion when the Mitrofanoff Principle is considered.