RESUMEN
BACKGROUND: The Italian Sarcoma Group and the Scandinavian Sarcoma Group designed a joint study to improve the prognosis for patients with Ewing's family tumors and synchronous metastatic disease limited to the lungs, or the pleura, or a single bone. PATIENTS AND METHODS: The study was opened in 1999 and closed to the enrollment in 2008. The program consisted of intensive five-drug combination chemotherapy, surgery and/or radiotherapy as local treatment, and consolidation treatment with high-dose busulfan/melphalan plus autologous stem cell rescue and total-lung irradiation. RESULTS: During the study period, 102 consecutive patients were enrolled. The median follow-up was 62 months (range 24-124). The 5-year event-free survival probability was 0.43 [standard deviation (SD) = 0.05] and the 5-year overall survival probability was 0.52 (SD = 0.052). Unfavorable prognostic factors emerging on multivariate analysis were a poor histological/radiological response at the site of the primary tumor [relative risk (RR) = 3.4], and incomplete radiological remission of lung metastases after primary chemotherapy (RR = 2.6). One toxic death and one secondary leukemia were recorded. CONCLUSIONS: This intensive approach is feasible and long-term survival is achievable in â¼50% of patients. New treatment approaches are warranted for patients responding poorly to primary chemotherapy.
Asunto(s)
Antineoplásicos/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/secundario , Neoplasias Pulmonares/secundario , Agonistas Mieloablativos/uso terapéutico , Sarcoma de Ewing/terapia , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Neoplasias Óseas/terapia , Busulfano/uso terapéutico , Niño , Preescolar , Terapia Combinada , Ciclofosfamida/uso terapéutico , Etopósido/uso terapéutico , Femenino , Humanos , Ifosfamida/uso terapéutico , Neoplasias Pulmonares/radioterapia , Neoplasias Pulmonares/terapia , Masculino , Melfalán/uso terapéutico , Metástasis de la Neoplasia/tratamiento farmacológico , Metástasis de la Neoplasia/radioterapia , Pronóstico , Sarcoma de Ewing/tratamiento farmacológico , Sarcoma de Ewing/radioterapia , Sarcoma de Ewing/secundario , Trasplante de Células Madre , Vincristina/uso terapéutico , Adulto JovenAsunto(s)
Osteosarcoma/diagnóstico , Osteosarcoma/terapia , Adolescente , Fosfatasa Alcalina/sangre , Antígenos CD/sangre , Biopsia , Quimioterapia Adyuvante , Niño , Terapia Combinada , Europa (Continente)/epidemiología , Femenino , Estudios de Seguimiento , Humanos , Incidencia , L-Lactato Deshidrogenasa/sangre , Masculino , Metástasis de la Neoplasia , Estadificación de Neoplasias , Osteosarcoma/patología , Reacción del Ácido Peryódico de Schiff , Pronóstico , Radioterapia , Medición de Riesgo , Prevención Secundaria , Resultado del TratamientoRESUMEN
BACKGROUND: The purpose of this study was to evaluate late effects and symptom complaints in long-term survivors (>5 years) of Extremity Bone Sarcoma (EBS survivors). The results were compared with findings in age- and gender-matched individuals from the general population (NORMs). PATIENTS AND METHODS: Among 155 EBS survivors approached, 133 (86%) were included, and 110 of them (83%) attended an outpatient examination. Health status was evaluated by a mailed questionnaire concerning demographic and current health issues, and physical examinations at the outpatient clinic. Age- and gender-adjusted normative controls were drawn from participants of the Health Study of Nord-Trøndelag County (HUNT 2). RESULTS: Median age at follow-up was 29 (15-57) years. Median follow-up was 12 (6-22) years. Of EBS survivors 42% had > or =1 somatic disease, 33% had ototoxicity and 13% had reduced renal function. EBS survivors were more likely to have heart disease (odds ratio [OR], 7.9; 95% confidence interval [95% CI], 2.5-25.3; P = 0.001), hypertension (OR, 3.4; 95% CI, 1.1-10.1; P = 0.03) and thyroid disease (OR, 3.0; 95% CI, 1.1-8.3; P = 0.04) compared to NORMs. EBS survivors reported more diarrhoea (29% vs. 19%, P = 0.02), palpitations (23% vs. 13%, P = 0.01) and shortness of breath (11% vs. 5%, P = 0.01) than NORMs. CONCLUSIONS: EBS survivors have poorer health status compared to age- and gender-matched controls. Long-term follow-up of these patients is therefore mandatory.
Asunto(s)
Neoplasias Óseas/epidemiología , Osteosarcoma/epidemiología , Sarcoma de Ewing/epidemiología , Sobrevivientes/estadística & datos numéricos , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/sangre , Neoplasias Óseas/terapia , Estudios de Casos y Controles , Quimioterapia Adyuvante , Comorbilidad , Diabetes Mellitus/diagnóstico , Diabetes Mellitus/epidemiología , Extremidades , Femenino , Estudios de Seguimiento , Estado de Salud , Cardiopatías/diagnóstico , Cardiopatías/epidemiología , Humanos , Masculino , Persona de Mediana Edad , Noruega/epidemiología , Osteosarcoma/sangre , Osteosarcoma/terapia , Cuidados Posoperatorios/estadística & datos numéricos , Radioterapia Adyuvante , Sarcoma de Ewing/sangre , Sarcoma de Ewing/terapia , Suecia/epidemiología , Enfermedades de la Tiroides/diagnóstico , Enfermedades de la Tiroides/epidemiología , Adulto JovenRESUMEN
The Scandinavian Sarcoma Group (SSG) started its first chemotherapy study in soft tissue sarcoma (STS) in 1981 (SSG I). This study evaluated single agent doxorubicin given adjuvant in a prospective randomized trial in patients with high-grade STS. Neither overall survival nor disease-free survival was improved. Combination chemotherapy was hereafter studied in a phase II study (1991-1994) combining ifosfamide and continuous infusion etoposide with growth factor support (SSG X). The response rate in previously untreated patients was high (42%), but complete remissions were few. Analysis made on patients operated after chemotherapy indicated improved survival in this subgroup. Meta-analyses of adjuvant chemotherapy for localised resectable STS in adults, including the SSG I trial, has indicated improved disease-free survival and a trend towards improved overall survival. Presently, SSG is testing whether such a benefit can be found for adjuvant ifosfamide and doxorubicin treatment given after primary surgery in selected patients with high-grade STS and other well defined unfavourable prognostic factors (SSG XIII).