RESUMEN
Acute lymphoblastic leukaemia (ALL) is the most common childhood malignancy. The survival rate in the Scandinavian countries is now around 85 %. ALL patients treated with cranial radiotherapy (CRT) are at risk for growth hormone deficiency (GHD), but little is known about other pituitary insufficiencies, e.g. ACTH. Adult ALL patients (median age at study 25 years), treated with 24 Gy (18-30) of CRT during childhood were investigated. We performed an insulin tolerance test (ITT) to evaluate cortisol secretion. We measured basal serum ACTH and cortisol levels before and after 5 years of GH therapy. 14 out of 37 (38 %) ALL patients had a subnormal cortisol response to an ITT (257-478 nmol/L) while there was no significant difference in basal cortisol levels between 44 patients and controls (P > 0.3). Female, but not male ALL patients had significantly lower ACTH levels compared to controls (P = 0.03). After 5 years of GH therapy only male ALL patients had significantly lowered basal plasma cortisol (P = 0.02). ALL survivors, treated with a moderate dose CRT, have a central adrenal insufficiency 20 years after diagnosis. An increased awareness of the risk for an adrenal insufficiency is of importance and life-long surveillance of the entire hypothalamic-pituitary axis is recommended in these patients.
Asunto(s)
Insuficiencia Suprarrenal/etiología , Leucemia-Linfoma Linfoblástico de Células Precursoras/radioterapia , Adolescente , Insuficiencia Suprarrenal/tratamiento farmacológico , Hormona Adrenocorticotrópica/sangre , Adulto , Niño , Preescolar , Femenino , Hormona del Crecimiento/deficiencia , Hormona de Crecimiento Humana/uso terapéutico , Humanos , Hidrocortisona/metabolismo , Sistema Hipotálamo-Hipofisario/fisiología , Lactante , Insulina , Factor I del Crecimiento Similar a la Insulina/metabolismo , Masculino , Sistema Hipófiso-Suprarrenal/fisiología , SobrevivientesRESUMEN
BACKGROUND: High-dose chemotherapy (HDT) was added to conventional chemotherapy in Ewing sarcoma family tumor (EFT) patients, poor responders (PRs) to induction chemotherapy in order to improve their survival. PATIENTS AND METHODS: Patients aged ≤40 years with nonmetastatic Ewing sarcoma (ES) received vincristine (V), doxorubicin (A), cyclofosfamide (C), actinomycin (Ac), ifosfamide (I) and etoposide (E) (VACAc-IE regimen) as induction chemotherapy. As maintenance treatment, good responders (GR) received nine cycles of VACAc-IE regimen. PRs received three cycles of VAC-IE, mobilizing cycle with CE and HDT with Busulfan and Melphalan with stem cell support. RESULTS: Three hundred patients [median age 15 years (3-40 years)] entered the study. One patient refused local treatment, 242 (81%) underwent surgery [with radiotherapy (RT) in 80] and 57 (19%) RT alone. No toxic deaths were recorded. Overall GR were 146 (49%). Twenty-eight PR did not receive HDT. At a median follow-up of 64 months (21-116 months), 5-year overall and event-free survival (EFS) were 75% and 69%, respectively. Five-year EFS was 75% for GR, 72% for PR treated with HDT and 33% for PR who did not receive HDT. CONCLUSIONS: High-dose therapy added to the VACA-IE regimen in PR patients is feasible and effective. Selected groups of patients with ES can benefit from HDT.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/terapia , Trasplante de Células Madre de Sangre Periférica , Sarcoma de Ewing/terapia , Adolescente , Adulto , Neoplasias Óseas/mortalidad , Busulfano/uso terapéutico , Niño , Preescolar , Ciclofosfamida/uso terapéutico , Dactinomicina/uso terapéutico , Supervivencia sin Enfermedad , Doxorrubicina/uso terapéutico , Etopósido/uso terapéutico , Femenino , Humanos , Ifosfamida/uso terapéutico , Estimación de Kaplan-Meier , Masculino , Melfalán/uso terapéutico , Agonistas Mieloablativos/uso terapéutico , Sarcoma de Ewing/mortalidad , Vincristina/uso terapéutico , Adulto JovenRESUMEN
The aim of the study was to illuminate the families' lived experience after completing a child's cancer treatment. The study took place at a University Hospital in southern Sweden. Interviews were carried out with 10 mothers, eight fathers, four patients and two siblings from a total of 10 families. The interviews were analysed with a hermeneutical phenomenological approach. One essential theme emerged from their stories, 'returning to a changed ordinary life--incorporating a trying and contradictory experience'. The families felt relieved that the treatment was over yet they experienced strains in their daily life. Family members felt changed and especially the parents needed to focus on themselves in order to recover. Closeness with other people, especially their own family, was important. The previously sick children felt a loss of concern from their parents when treatment had ended, in contrast to siblings who experienced increased attention from their parents. Parents experienced being in uncharted territory and sometimes missed the security of hospital. For professionals it is important to offer the family a structured follow-up to help them in their daily life after the child's treatment is completed.
Asunto(s)
Cuidadores/psicología , Familia/psicología , Neoplasias/psicología , Adulto , Niño , Preescolar , Salud de la Familia , Femenino , Humanos , Lactante , Masculino , Persona de Mediana Edad , Neoplasias/terapia , Psicología Infantil , Aislamiento Social , Apoyo Social , Estrés Psicológico/etiología , Encuestas y Cuestionarios , SueciaRESUMEN
Childhood cancer survivors (CCS) have an increased risk of impaired spermatogenesis, but data regarding the disease- and treatment-related risk factors of azoospermia are scarce. Such information is crucial both for counselling CCS and for selecting patients for testicular tissue cryopreservation. The proportion of azoospermic men in CCS was 18% [95% confidence interval (CI): 12-26], specifically for leukaemias (19%; 95% CI: 5.5-42), Hodgkin's disease (53%; 95% CI: 29-76), non-Hodgkin's lymphoma (11%; 95% CI: 0.28-48) and testicular cancer (11%; 95% CI: 0.28-48). In CCS treated with high doses of alkylating agents, the proportion of azoospermic men was 80% (95% CI: 28-99) and if radiotherapy was used additionally, the proportion was 64% (95% CI: 35-87). In CCS with subnormal Inhibin B levels, the proportion of azoospermic men was 66% (95% CI: 47-81) and for those with elevated follicle-stimulating hormone (FSH) levels, the proportion was 50% (95% CI: 35-67). Among CCS with subnormal testicular volume (≤ 24 mL), azoospermia was found in 61% (95% CI: 39-80) of the cases. Most childhood cancer diagnoses are associated with an increased risk of azoospermia, especially in CCS receiving testicular irradiation, high doses of alkylating drugs and other types of cytotoxic treatment, if combined with irradiation. Inhibin B, FSH and testicular volume can be used as predictors for the risk of azoospermia.
Asunto(s)
Alquilantes/efectos adversos , Azoospermia/etiología , Neoplasias/terapia , Adulto , Antineoplásicos Alquilantes , Azoospermia/epidemiología , Causalidad , Niño , Ensayo de Inmunoadsorción Enzimática , Hormona Folículo Estimulante/sangre , Enfermedad de Hodgkin/terapia , Humanos , Inhibinas/sangre , Leucemia/terapia , Linfoma no Hodgkin/terapia , Masculino , Persona de Mediana Edad , Neoplasias/tratamiento farmacológico , Neoplasias/radioterapia , Pubertad , Sobrevivientes , Neoplasias Testiculares/etiología , Neoplasias Testiculares/terapiaAsunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/tratamiento farmacológico , Sarcoma de Ewing/tratamiento farmacológico , Neoplasias Óseas/mortalidad , Neoplasias Óseas/radioterapia , Neoplasias Óseas/cirugía , Protocolos Clínicos , Humanos , Estudios Multicéntricos como Asunto , Análisis Multivariante , Pronóstico , Radioterapia Adyuvante , Ensayos Clínicos Controlados Aleatorios como Asunto , Sistema de Registros , Inducción de Remisión , Sarcoma de Ewing/mortalidad , Sarcoma de Ewing/radioterapia , Sarcoma de Ewing/cirugía , Análisis de Supervivencia , Resultado del TratamientoAsunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/tratamiento farmacológico , Osteosarcoma/tratamiento farmacológico , Neoplasias Óseas/mortalidad , Protocolos Clínicos , Supervivencia sin Enfermedad , Humanos , Interferón-alfa/uso terapéutico , Estudios Multicéntricos como Asunto , Osteosarcoma/mortalidad , Ensayos Clínicos Controlados Aleatorios como Asunto , Países Escandinavos y NórdicosRESUMEN
From 1990 to 1997, 113 eligible patients with classical osteosarcoma received neo-adjuvant chemotherapy consisting of high-dose methotrexate, cisplatin and doxorubicin. Good histological responders continued to receive the same therapy postoperatively, while poor responders received salvage therapy with an etoposide/ifosfamide combination. With a median follow-up of 83 months, the projected metastasis-free and overall survival rates at 5 years are 63 and 74%, respectively. Independent favourable prognostic factors for outcome were tumour volume < 190 ml, 24-h serum methotrexate > 4.5 microM and female gender. The etoposide/ifosfamide replacement combination did not improve outcome in the poor histological responders. In conclusion, this intensive multi-agent chemotherapy results in > 70% of patients with classical osteosarcoma surviving for 5 years. The data obtained from this non-randomised study do not support discontinuation and exchange of all drugs used preoperatively in histological poor responders. As observed in previous Scandinavian osteosarcoma studies, female gender appears to be a strong predictor of a favourable outcome.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/tratamiento farmacológico , Osteosarcoma/tratamiento farmacológico , Terapia Recuperativa , Adolescente , Adulto , Anciano , Neoplasias Óseas/patología , Neoplasias Óseas/cirugía , Quimioterapia Adyuvante , Niño , Preescolar , Cisplatino/administración & dosificación , Doxorrubicina/administración & dosificación , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Metotrexato/administración & dosificación , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Osteosarcoma/patología , Osteosarcoma/cirugía , Cooperación del Paciente , Pronóstico , Análisis de SupervivenciaRESUMEN
The first Scandinavian protocol for Ewing's sarcoma, SSG IV, resulted in a local control rate of 74% and 5-year metastasis-free survival (MFS) of 43%. The second protocol, SSG IX, was started in order to improve upon these results. It featured four chemotherapy cycles, each consisting of two courses of VAI (vincristine, doxorubicin, ifosfamide) alternating with one course of PAI (cisplatin, doxorubicin, ifosfamide) at 3-weekly intervals. Total treatment time was 35 weeks. Local therapy was given at week 9. Inoperable or non-radically operated patients received hyperfractionated accelerated radiotherapy 1.5 Gy twice daily between chemotherapy courses to a total dose of 42-60 Gy, depending on surgical radicality and tumour localisation. 88 patients were included (58 male, 30 female, mean age 20 years; range 5-65 years). The tumour (73 M0 and 15 M1) was located centrally in 31 patients (35%), in the extremities in 34 (39%) and other sites in 23 (26%) of cases. The median size of tumour was 10 cm (range 2-23), soft tissue was invaded in 87%. Surgery was the local therapy for 60 (68%) patients: amputation in 8 and local excision in 52. The surgical margins were wide in 35 patients, marginal in 14 and intralesional in 3. Radiotherapy was given to 17 non-radically operated patients postoperatively and to 28 patients with inoperable tumours primarily. Histological responses were evaluated in 52 patients. 9 local recurrences were observed (10%). Distant metastases developed in 24 M0 patients (33%). The estimated 5-year MFS was 58% and overall survival (OS) 70% for M0 and 27% and 28% for M1 patients, respectively. Survival was favourable in patients with non-metastatic extremity tumours (90%) and tumours operated with wide margins (90%). Patients with a total necrosis after chemotherapy had a better OS than those with a partial or poor response (P=0.003). The toxicity (World Health Organisation) was acceptable (gastrointestinal G1-2; haematological G3-4). The SSG IX protocol gave better local control and survival rates than the SSG IV. Whether this is due to a higher therapeutic efficacy of the present protocol cannot be ascertained in this comparison with a historical control.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/tratamiento farmacológico , Sarcoma de Ewing/tratamiento farmacológico , Adolescente , Adulto , Anciano , Neoplasias Óseas/radioterapia , Niño , Preescolar , Cisplatino/uso terapéutico , Terapia Combinada , Doxorrubicina/uso terapéutico , Femenino , Humanos , Ifosfamida/uso terapéutico , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Sarcoma de Ewing/radioterapia , Tasa de Supervivencia , Factores de Tiempo , Resultado del Tratamiento , Vincristina/uso terapéuticoRESUMEN
BACKGROUND: Hepatoblastoma (HBT) is the most common hepatic neoplasm in children. This notwithstanding, little is known about pathogenetic factors, such as genetic abnormalities, of importance for the development and progression of this tumor type. To date, only 33 cytogenetically abnormal HBT have been published, and trisomies for chromosomes 2 and 20 have been shown to be the most frequent aberrations. Recently, unbalanced translocations involving proximal 1q have been described in several HBT, suggesting that a pathogenetically important gene maps to 1q. PROCEDURE: Six primary and one recurrent HBT were cytogenetically analyzed after short-term tissue culture. In addition, fluorescence in situ hybridization (FISH) studies, using locus-specific probes, were performed on three of these pediatric HBT as well as on one previously reported adult HBT. RESULTS: Total or partial trisomy 8, gain of chromosome 20, and structural rearrangements of chromosome 1 were detected in three HBT, and overrepresentation of chromosome 2 material was found in two HBT. The adjacent chromosome bands 1q12 and 1q21 were involved in three translocations, t(1;2), t(1;4), and t(1;11), which were all unbalanced and resulted in gain of 1q material. The previously reported adult HBT displayed 1q deletions with breakpoints at 1q12-21. FISH analyses of the 1q rearrangements revealed that all breakpoints were within the heterochromatic region. CONCLUSIONS: These findings provide further support for the importance of trisomies 2, 8, and 20 and rearrangements of 1q in the development of HBT. Furthermore, the consistent localization of breakpoints within the heterochromatic segment of chromosome 1 suggests that the important pathogenetic consequence of 1q abnormalities is the resulting genomic imbalance rather than a specific gene rearrangement.
Asunto(s)
Cromosomas Humanos Par 1/genética , Reordenamiento Génico/genética , Hepatoblastoma/genética , Neoplasias Hepáticas/genética , Preescolar , Bandeo Cromosómico , Cromosomas Humanos Par 20/genética , Cromosomas Humanos Par 8/genética , Femenino , Humanos , Hibridación Fluorescente in Situ , Lactante , Cooperación Internacional , Cariotipificación , Masculino , Trisomía/genéticaRESUMEN
Twenty-eight cases of hepatitis C virus (HCV) infection were identified in children in a pediatric oncology ward during 2 nosocomial outbreaks. HCV infection spontaneously cleared in 6 patients (21%). Eleven patients with persistent HCV viremia who had malignant diseases in remission after treatment were given a 48-week course of combined therapy with interferon-alpha (5x106 U 3 times weekly) and oral ribavirin (15 mg/kg/d). Seven (64%) of the 11 patients had sustained virological responses 6 and 12 months after cessation of therapy. Side effects were common but generally were mild or moderate.
Asunto(s)
Antivirales/uso terapéutico , Hepatitis C/tratamiento farmacológico , Interferón-alfa/uso terapéutico , Neoplasias/terapia , Ribavirina/uso terapéutico , Adolescente , Adulto , Niño , Preescolar , Infección Hospitalaria , Brotes de Enfermedades , Quimioterapia Combinada , Hepatitis C/complicaciones , Humanos , Neoplasias/complicaciones , Inducción de RemisiónRESUMEN
Three childhood acute monoblastic leukemias (AML M5) with granulocytic sarcomas (GSs) are described. All displayed 11q23/MLL abnormalities, t(9;11)(p22;q23) in two cases and t(11;17)(q23;q21) in one case, constituting around 20% of all 11q23-positive AML cytogenetically investigated in our department. Two of the patients had GS in multiple locations, and all three had abdominal GS. In two of them, t(9;11)-positive GS was diagnosed prior to the diagnosis of AML. Fourteen (1.9%) of 752 published AML cases with 11q23 aberrations have had GS, either as a presenting feature or during disease progression. The incidence of GS has varied significantly (P < 0.05) between children (3.8%) and adults (0.8%). The most common AML subtype has been AML M5 ( approximately 75%) and the most frequent GS sites have been the skin, abdomen, orbit, and thorax. Considering the possibility of underreporting of GS in published cases and the relatively high frequency in our own series, we believe that 11q23/MLL rearrangements may predispose to GS development. Although extramedullary infiltrates in the skin are known to be frequent in cases of AML M5, which is often associated with 11q23 aberrations, the present findings indicate that GS in the abdomen, orbit, and thorax may also be common, especially in pediatric AML. Thus, the possibility of 11q23/MLL-positive GS should be suspected when tumors of uncertain derivation occur in these sites. Finally, the identification of 11q23/MLL abnormalities in GSs in two patients without overt AML underscores the importance of using cytogenetic and molecular genetic investigations as a diagnostic approach in the evaluation of tumorous lesions of unknown origin. Genes Chromosomes Cancer 27:136-142, 2000.
Asunto(s)
Cromosomas Humanos Par 11/genética , Proteínas de Unión al ADN/genética , Leucemia Monocítica Aguda/genética , Leucemia Mieloide/genética , Proto-Oncogenes , Factores de Transcripción , Translocación Genética , Abdomen , Adolescente , Anciano , Southern Blotting , Niño , Análisis Citogenético , ADN de Neoplasias/análisis , ADN de Neoplasias/genética , Femenino , Reordenamiento Génico , N-Metiltransferasa de Histona-Lisina , Humanos , Lactante , Recién Nacido , Leucemia Monocítica Aguda/patología , Leucemia Mieloide/patología , Masculino , Proteína de la Leucemia Mieloide-LinfoideRESUMEN
During the past 15 years the Scandinavian Sarcoma Group has treated 140 patients with Ewing's sarcoma. Two protocols have been used. SSG IV included 52 patients between 1984 and 1990 and SSG IX, 88 patients since 1990. After 5 years of treatment, local recurrences occurred in 19% of the patients (M0 + M1) in the SSG IV group and 10% in the SSG IX group. Distant metastases developed in 57% of the M0-patients in the SSG IV group and in 33% in the SSG IX group. Tumor-related survival (overall) of M0-patients was 49% in SSG IV and 70% in SSG IX, and the metastasis-free survival rate 45% and 58%, respectively. Patients having a localized extremity tumor had a survival rate of 90% (SSG IX). In both treatment groups, good responders to chemotherapy had a better survival rate than poor ones (SSG IV, p < 0.02, GI-II vs. G II-IV and SSG IX, p < 0.003, GI-III vs. G IV). In conclusions local control and survival rates were better with SSG IX than SSG IV.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/tratamiento farmacológico , Recurrencia Local de Neoplasia/patología , Sarcoma de Ewing/tratamiento farmacológico , Adolescente , Adulto , Anciano , Neoplasias Óseas/mortalidad , Neoplasias Óseas/radioterapia , Neoplasias Óseas/cirugía , Niño , Preescolar , Terapia Combinada , Supervivencia sin Enfermedad , Humanos , Persona de Mediana Edad , Estadificación de Neoplasias , Pronóstico , Sistema de Registros , Sarcoma de Ewing/mortalidad , Sarcoma de Ewing/radioterapia , Sarcoma de Ewing/cirugía , Países Escandinavos y Nórdicos/epidemiologíaAsunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/tratamiento farmacológico , Osteosarcoma/tratamiento farmacológico , Adolescente , Antimetabolitos Antineoplásicos/administración & dosificación , Neoplasias Óseas/mortalidad , Neoplasias Óseas/cirugía , Quimioterapia Adyuvante , Niño , Cisplatino/administración & dosificación , Supervivencia sin Enfermedad , Doxorrubicina/administración & dosificación , Femenino , Estudios de Seguimiento , Humanos , Masculino , Metotrexato/administración & dosificación , Osteosarcoma/mortalidad , Osteosarcoma/cirugía , Cuidados Posoperatorios/métodos , Cuidados Preoperatorios/métodos , Pronóstico , Sistema de Registros , Países Escandinavos y Nórdicos/epidemiologíaRESUMEN
BACKGROUND: Despite screening of blood donors, hepatitis C virus (HCV) infection can occur in patients who receive multiple transfusions. OBJECTIVE: To clarify mechanisms of nosocomial transmission of HCV. DESIGN: Epidemiologic and molecular analyses of hepatitis C outbreaks. SETTING: Pediatric oncology ward. PATIENTS: Children with cancer. MEASUREMENTS: Epidemiologic analysis, HCV RNA detection, genotyping, and hypervariable region 1 (HVR1) sequencing. RESULTS: Ten cases of infection with acute HCV genotype 3a occurred between 1990 and 1993. Sequencing of HVR1 revealed three related strains. Despite an overhaul of hygiene procedures, a patient infected with genotype 1b generated nine subsequent infected patients in 1994. Several patients had high virus titers and strongly delayed anti-HCV antibody responses. All had permanent intravenous catheters. Multidose vials used for flushing or treatment had probably been contaminated during periods of overlapping treatment. CONCLUSIONS: Contamination of multidose vials was the most likely mode of HCV transmission; therefore, use of such vials should be restricted. Rigorous adherence to hygiene routines remains essential to preventing transmission of bloodborne infections.
Asunto(s)
Brotes de Enfermedades , Hepacivirus/genética , Hepatitis C/epidemiología , Secuencia de Bases , Niño , Infección Hospitalaria/epidemiología , Infección Hospitalaria/transmisión , Infección Hospitalaria/virología , Transmisión de Enfermedad Infecciosa , Contaminación de Equipos , Genotipo , Hepacivirus/aislamiento & purificación , Hepatitis C/transmisión , Hepatitis C/virología , Anticuerpos contra la Hepatitis C/sangre , Humanos , Datos de Secuencia Molecular , Servicio de Oncología en Hospital , ARN ViralRESUMEN
BACKGROUND: Ewing sarcoma is associated with a nonrandom pattern of primary and secondary chromosomal aberrations. Whereas the finding of rearrangements of chromosome 22, usually in the form of a balanced translocation t(11;22)(q24;q12), is important diagnostically, nothing is known about the potential prognostic impact of the secondary chromosomal aberrations. PROCEDURE: During a 1 3-year-period, short-term cultured tumor samples from 21 children and young adults with Ewing sarcoma were cytogenetically analyzed successfully. RESULTS: Clonal chromosome aberrations were detected in 18 patients, 17 of whom had the characteristic t(11;22)(q24;q12) or variants thereof. The most frequent secondary change was +8, followed by +12, +2, +5, +9, +15, and gain of material from the long and short arms of chromosome 1. The only recurrent secondary change that was restricted to tumors from the ten patients that were dead at latest follow-up was gain of 1q material. Furthermore, all three patients with tumors with chromosome numbers over 50 had died, and the only patient with a tumor karyotype lacking chromosome 22 rearrangement was alive without evidence of disease. CONCLUSIONS: These data and previously published results indicate that the karyotypic pattern not only may be of diagnostic significance but also may be important prognostically.
Asunto(s)
Neoplasias Óseas/genética , Neoplasias Óseas/patología , Aberraciones Cromosómicas/genética , Sarcoma de Ewing/genética , Sarcoma de Ewing/patología , Adolescente , Adulto , Neoplasias Óseas/terapia , Niño , Trastornos de los Cromosomas , Mapeo Cromosómico , Femenino , Humanos , Cariotipificación , Masculino , Sarcoma de Ewing/terapia , Translocación Genética/genética , Resultado del TratamientoRESUMEN
During a 16-year period, 49 children were treated for Wilms' tumor (WT); 7 were bilateral, 5 synchronous and 2 metachronous. The age at primary diagnosis was 6 months to 5 years (mean 2.4 years). All but 1 child received preoperative chemotherapy with tumor reduction. Unilateral nephrectomy was performed in 2 metachronous cases. In 3 synchronous WTs, the tumor was enucleated in 5 kidneys and a heminephrectomy was performed in 1 kidney with a double pelvis. Two children were not operated upon. At relapse in the contralateral kidney the tumor was enucleated. Three patients died of WT, 1 is alive with disease, and 3 are without evidence of disease. Renal salvage procedures were technically feasible without complications and are advocated to preserve renal function.
Asunto(s)
Neoplasias Renales/cirugía , Neoplasias Primarias Múltiples/cirugía , Neoplasias Primarias Secundarias/cirugía , Tumor de Wilms/cirugía , Antineoplásicos/uso terapéutico , Preescolar , Terapia Combinada , Femenino , Humanos , Lactante , Neoplasias Renales/tratamiento farmacológico , Masculino , Neoplasias Primarias Múltiples/tratamiento farmacológico , Neoplasias Primarias Secundarias/tratamiento farmacológico , Nefrectomía , Resultado del Tratamiento , Tumor de Wilms/tratamiento farmacológicoRESUMEN
A report on the long-term follow up of the first cooperative Scandinavian Sarcoma Group study in Ewing's sarcoma of bone is presented. Fifty-two previously untreated patients entered the study between 1984 and 1990. Half of the tumors were located in the extremities and one quarter in the pelvis. The combined modality treatment consisted of 5 cycles of chemotherapy--including vincristine, methotrexate, doxorubicin, cyclophosphamide, bleomycin and dactinomycin. The first two cycles were followed by local resection or amputation in 35 patients and by radiotherapy alone in 17 patients. When surgery was not performed, was incomplete or yielded poor margins radiotherapy was given at a dose of 40-60 Gy. Local tumor relapses developed in 10 patients and in all but one patient were accompanied by metastatic disease. Five patients had metastasis at diagnosis and distant metastases developed after primary treatment in 27 patients after a median of 14 months. The median follow-up time for the 20 surviving patients is 10 years. At 5 years the tumor-related survival was 46% and the metastasis-free survival 43%. Late tumor relapses occurred in 4 patients, which reduced the 10-year tumor related survival to 41% and the metastasis-free survival to 38%. Histopathological tumour response correlated with survival with 5-year metastasis-free survival rates of 73% for the good responders and 35% for the poor responders.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/terapia , Sarcoma de Ewing/terapia , Adolescente , Adulto , Bleomicina/administración & dosificación , Neoplasias Óseas/patología , Neoplasias Óseas/radioterapia , Neoplasias Óseas/cirugía , Niño , Terapia Combinada , Ciclofosfamida/administración & dosificación , Dactinomicina/administración & dosificación , Doxorrubicina/administración & dosificación , Esquema de Medicación , Femenino , Estudios de Seguimiento , Humanos , Masculino , Metotrexato/administración & dosificación , Recurrencia Local de Neoplasia , Procedimientos Ortopédicos , Radioterapia Adyuvante , Sarcoma de Ewing/patología , Sarcoma de Ewing/radioterapia , Sarcoma de Ewing/cirugía , Países Escandinavos y Nórdicos , Análisis de Supervivencia , Resultado del Tratamiento , Vincristina/administración & dosificaciónRESUMEN
During a 15-y period, 48 children were treated for Wilms' tumour (WT). Seven of them were < 1 y of age at diagnosis. One child presented with non-traumatic haematuria, but in all the other children WT was revealed as a palpable abdominal mass at routine examination or investigation due to another disease. The four children under 6 months of age at diagnosis were primarily operated upon; the others received preoperative chemotherapy. Two children had chromosomal aberrations in the WT tumour specimen. The follow-up revealed that postoperatively six children are healthy with no evidence of relapse from WT, but one child had a contralateral relapse successfully enucleated. The clinical behaviour and management of WT in infants differ compared with that in older children. The diagnosis may be uncertain and it can be difficult to distinguish between malignant and non-malignant lesions. It is essential to realize the possibility of WT, even in children < 1 y of age.
Asunto(s)
Neoplasias Renales , Tumor de Wilms , Aberraciones Cromosómicas , Femenino , Humanos , Lactante , Neoplasias Renales/diagnóstico , Neoplasias Renales/genética , Neoplasias Renales/terapia , Masculino , Estudios Retrospectivos , Tumor de Wilms/diagnóstico , Tumor de Wilms/genética , Tumor de Wilms/terapiaRESUMEN
In 26 consecutive patients operated for Wilms' tumour samples from the tumour were genetically analyzed. Clonal acquired chromosome aberrations were found in 13 patients and a constitutional trisomy 18 as the sole change in 1. The chromosome number was altered in 13 patients. Numerical changes occurred in 16 patients and breakpoint of chromosome 1 in 6 patients. There was no structural alteration of chromosome 11. The observed cytogenetic heterogeneity illustrates the complexity of genetic changes involved in the genesis and progression of Wilms' tumour. To further elucidate the phenotypic impact of chromosomal aberrations the correlation to histology and the clinical course will be important.
Asunto(s)
Aberraciones Cromosómicas , Tumor de Wilms/genética , Niño , Preescolar , Femenino , Heterogeneidad Genética , Humanos , Lactante , MasculinoRESUMEN
This study provides a comparative cost-effectiveness analysis of three universal immunization programs for hepatitis B virus (HBV). Using three theoretical cohorts of infants, 10-year-olds, and 12-year-olds, a universal immunization program was compared with a prenatal screening/newborn immunization program involving testing of prepartum women and immunization of newborns of HBsAg-positive mothers. A Markov long-term outcome model used Manitoba data to estimate costs and health outcomes across the lifespan. The model was based on an HBV incidence rate of 19/100,000 and a discount rate of 5% and incorporated the most recent treatment advances (interferon therapy). Cost-effectiveness was calculated as the ratio of dollars spent per year of life saved, with costs determined from the perspective of a third-party payer. The universal infant-immunization program, although not cost-saving, was associated with a low, economically attractive cost-effectiveness ratio of $15,900 (Canadian) per year of life saved, a figure substantially lower than the ratios of $97,600 and $184,800 (Canadian) associated with the universal programs for 10- and 12-year-olds, respectively. Cost-effectiveness ratios were found to be sensitive to changes in immunization costs, HBV incidence rates, and the rate at which protective antibody levels are lost over time: If these variables move in the directions suggested by current trends, the authors anticipate an increasing economic appeal of universal programs well into the future. A universal program of HBV immunization for infants appears to be economically practical in regions where HBV infection rates are low and stable.