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1.
Lancet Oncol ; 19(8): 1061-1071, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29941280

RESUMO

BACKGROUND: Rhabdomyosarcoma is an aggressive tumour that can develop in almost any part of the body. Doxorubicin is an effective drug against rhabdomyosarcoma, but its role in combination with an established multidrug regimen remains controversial. Therefore, we aimed to evaluate the possible benefit of early dose intensification with doxorubicin in patients with non-metastatic rhabdomyosarcoma. METHODS: We did a multicentre, open-label, randomised controlled, phase 3 trial involving 108 hospitals from 14 countries. We included patients older than 6 months but younger than 21 years with a pathologically proven diagnosis of rhabdomyosarcoma. We assigned each patient to a specific subgroup according to the EpSSG stratification system. Those with embryonal rhabdomyosarcoma incompletely resected and localised at unfavourable sites with or without nodal involvement, or those with alveolar rhabdomyosarcoma without nodal involvement were considered at high risk of relapse. These high-risk patients were randomly assigned (1:1) to receive either nine cycles of IVA (ifosfamide 3 g/m2 given as a 3-h intravenous infusion on days 1 and 2, vincristine 1·5 mg/m2 weekly during the first 7 weeks then only on day 1 of each cycle [given as a single intravenous injection], and dactinomycin 1·5 mg/m2 on day 1 given as a single intravenous injection) or four cycles of IVA with doxorubicin 30 mg/m2 given as a 4-h intravenous infusion on days 1 and 2 followed by five cycles of IVA. The interval between cycles was 3 weeks. Randomisation was done using a web-based system and was stratified (block sizes of four) by enrolling country and risk subgroup. Neither investigators nor patients were masked to treatment allocation. The primary endpoint was 3-year event-free survival assessed by the investigator at each centre in the intention-to-treat population. Patients who received at least one dose of study treatment were considered in the safety analysis. In agreement with the independent data monitoring committee, the study was closed to patient entry on Dec 16, 2013, after futility analysis. This trial is registered with EudraCT, number 2005-000217-35, and is currently in follow-up. FINDINGS: Between Oct 1, 2005, and Dec 16, 2013, 484 patients were randomly assigned to receive each chemotherapy regimen (242 in the IVA group and 242 in the IVA plus doxorubicin group). Median follow-up was 63·9 months (IQR 44·6-78·9). The 3-year event-free survival was 67·5% (95% CI 61·2-73·1) in the IVA plus doxorubicin group and 63·3% (56·8-69·0) in the IVA group (hazard ratio 0·87, 95% CI 0·65-1·16; p=0·33). Grade 3-4 leucopenia (232 [93%] of 249 patients in the IVA plus doxorubicin group vs 194 [85%] of 227 in the IVA group; p=0·0061), anaemia (195 [78%] vs 111 [49%]; p<0·0001), thrombocytopenia (168 [67%] vs 59 [26%]; p<0.0001), and gastrointestinal adverse events (78 [31%] vs 19 [8%]; p<0·0001) were significantly more common in the IVA plus doxorubicin group than in the IVA group. Grade 3-5 infections (198 [79%] vs 128 [56%]; p<0·0001) were also significantly more common in the IVA plus doxorubicin group than in the IVA group, in which one patient had grade 5 infection. Two treatment-related deaths were reported (one patient developed septic shock and one affected by Goldenhar syndrome developed intractable seizures) in the IVA plus doxorubicin group, both occurring after the first cycle of treatment, and none were reported in the IVA group. INTERPRETATIONS: The addition of dose-intensified doxorubicin to standard IVA chemotherapy did not show a significant improvement in the outcome of patients with high-risk non-metastatic rhabdomyosarcoma. Therefore, the IVA chemotherapy regimen should remain the standard of care for patients with localised rhabdomyosarcoma in Europe. FUNDING: Fondazione Città della Speranza, Italy, and the Association Léon Berard Enfant Cancéreux, France.

2.
Pediatr Blood Cancer ; 65(6): e26974, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29350487

RESUMO

INTRODUCTION: Cutaneous melanoma is rare in childhood and published studies have mainly been retrospective single-institution series or small case series. Given the absence of clinical protocols dedicated to pediatric melanoma, the treatment approach is generally extrapolated from the ones applied to adults. METHODS: Coordinated by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT), this study collected patients prospectively registered between 2002 and 2012 under national cooperative projects dedicated to rare pediatric tumors in Italy, Poland, Germany, and France. Additional cases were collected from dermatology registries in Germany and Israel. RESULTS: A total of 219 patients aged 0-18 years (median 14.4) were included in the analysis. Sentinel lymph node biopsy was performed in 112 patients (76% of those with Breslow thickness > 0.75 mm) and was positive in 37.5%. Systemic therapy was used in 33 cases. In stage III cases, survival rates were similar for patients who received (23 cases) or not (21 cases) adjuvant therapy. For the whole series, 3-year overall and disease-free survival rates were 91.4% and 84.0%, respectively (median follow-up 41.8 months). Tumor site, tumor stage, and ulceration influenced survival rates. Patients treated by pediatric oncologists (n = 140) were more likely to have advanced disease than those treated by dermatologists (n = 79). DISCUSSION: This study would suggest that the clinical history of melanoma in children and adolescents might resemble that of adult counterpart. Cooperative efforts are needed to make new drugs more readily available to pediatric patients to increase the outcome of patient with advanced disease.

4.
Eur J Cancer ; 81: 206-225, 2017 08.
Artigo em Inglês | MEDLINE | ID: mdl-28649001

RESUMO

BACKGROUND: The use of chemotherapy to manage newly diagnosed low grade glioma (LGG) was first introduced in the 1980s. One randomised trial has studied two- versus four-drug regimens with a duration of 12 months of treatment after resection. METHODS: Within the European comprehensive treatment strategy for childhood LGG, the International Society of Paediatric Oncology-Low Grade Glioma (SIOP LGG) Committee launched a randomised trial involving 118 institutions and 11 countries to investigate the addition of etoposide (100 mg/m2, days 1, 2 & 3) to a four-course induction of vincristine (1.5 mg/m2 × 10 wkly) and carboplatin (550 mg/m2 q 3 weekly) as part of 18-month continuing treatment programme. Patients were recruited after imaging diagnosis, resection or biopsy with progressive disease/symptoms. Some 497 newly diagnosed patients (M/F 231/266; median age 4.26 years (interquartile range (IQR) 2.02-7.06)) were randomised to receive vincristine carboplatin (VC) (n = 249) or VC plus etoposide (VCE) during induction (n = 248), stratified by age and tumour site. FINDINGS: No differences between the two arms were found in term of survival and radiological response. Response and non-progression rates at 24 weeks for VC and VCE, were 46% versus 41%, and 93% versus 91% respectively; 5-year Progression-Free Survival (PFS) and Overall Survival (OS) were 46% (StDev 3.5) versus 45% (StDev 3.5) and 89% (StDev 2.1) versus 89% (StDev 2.1) respectively. Age and diencephalic syndrome are adverse clinical risk factors for PFS and OS. 5-year OS for patients in early progression at week 24 were 46% (StDev 13.8) and 49% (StDev 16.5) in the two arms, respectively. INTERPRETATION: The addition of etoposide to VC did not improve PFS or OS. High non-progression rates at 24 weeks justify retaining VC as standard first-line therapy. Infants with diencephalic syndrome and early progression need new treatments to be tested. Future trials should use neurological/visual and toxicity outcomes and be designed to discriminate between the impact on disease outcomes of 'duration of therapy' and 'age at stopping therapy'.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Encefálicas/tratamento farmacológico , Glioma/tratamento farmacológico , Quimioterapia de Indução/métodos , Carboplatina/administração & dosagem , Criança , Pré-Escolar , Etoposídeo/administração & dosagem , Feminino , Humanos , Lactente , Masculino , Análise de Sobrevida , Vincristina/administração & dosagem
5.
Clin Cancer Res ; 23(13): 3316-3324, 2017 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-28119362

RESUMO

Purpose: LKB1 is a key sensor of metabolic stress, including hypoxia and glucose deprivation, two features of the tumor microenvironment exacerbated by antiangiogenic therapy. We investigated the role of LKB1 as a potential predictive marker of sensitivity to bevacizumab in advanced non-small cell lung cancer (aNSCLC).Experimental design: We retrospectively analyzed LKB1 expression by IHC in 98 samples from 125 patients with aNSCLC, including 59 patients treated with chemotherapy and 39 treated with chemotherapy plus bevacizumab. IHC intensity was recoded in two classes (negative/weak vs. moderate/intense) and correlated with outcome according to treatment arm. Patient-derived tumor xenografts (PDXs) were used to investigate mechanisms involved in preclinical models.Results: In the whole study population (125), median OS and PFS were 11.7 [95% confidence interval (CI), 9.1-15.3] and 6.7 (95% CI, 5.7-7.2) months, respectively. Differential impact of the marker on outcome of the 98 patients was highlighted according to the treatment. Patients with negative/weak LKB1 status did not have a statistically significant benefit from bevacizumab added to chemotherapy (HR for patients treated with bevacizumab: 0.89; 95% CI, 0.51-1.56; P = 0.6803), whereas patients expressing moderate/intense LKB1 and receiving bevacizumab had significant lower risk of death compared with those not receiving bevacizumab (HR, 0.26; 95% CI, 0.10-0.64; P = 0.0035). Loss of LKB1 was associated with reduced AMPK activation in PDXs and increased tumor necrosis following bevacizumab administration, highlighting impaired control of the metabolic stress caused by this antiangiogenic drug.Conclusions: Our data hint at a possible predictive impact of LKB1 expression in patients with aNSCLC treated with chemotherapy plus bevacizumab. Clin Cancer Res; 23(13); 3316-24. ©2017 AACR.


Assuntos
Bevacizumab/administração & dosagem , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Neovascularização Patológica/tratamento farmacológico , Proteínas Serina-Treonina Quinases/genética , Idoso , Inibidores da Angiogênese/administração & dosagem , Animais , Carcinoma Pulmonar de Células não Pequenas/genética , Carcinoma Pulmonar de Células não Pequenas/patologia , Intervalo Livre de Doença , Feminino , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Humanos , Masculino , Camundongos , Pessoa de Meia-Idade , Neovascularização Patológica/genética , Neovascularização Patológica/patologia , Proteínas Serina-Treonina Quinases/antagonistas & inibidores , Ensaios Antitumorais Modelo de Xenoenxerto
6.
Oncotarget ; 8(4): 6433-6445, 2017 Jan 24.
Artigo em Inglês | MEDLINE | ID: mdl-28031535

RESUMO

Deregulated glucose metabolism is observed in cancer but whether this metabolic trait influences response to or is modulated by cytotoxic drugs is unknown. We show here that tumor cells from epithelial ovarian cancer (EOC) patients can be categorized, according to their in vitro viability under glucose starvation, into glucose deprivation-sensitive (glucose-addicted, GA) and glucose deprivation-resistant (glucose non-addicted, GNA). When EOC cells were cultured in the absence of glucose, all samples from platinum (PLT)-sensitive patients felt into the GA group; they disclosed higher expression of glucose metabolism enzymes, higher proliferation rates and in vitro sensitivity to PLT. Moreover, GA patients showed reduced multi-drug resistance pump expression and autophagy, compared to GNA samples. The close association between PLT sensitivity and glucose metabolic profile was confirmed in a xenograft model, where a stringent parallelism between PLT sensitivity/resistance and glucose metabolism was identified. Finally, in a cohort of naïve EOC patients categorized as GA or GNA at diagnosis, Kaplan Meier curves showed that the GA phenotype was associated with significantly better progression-free survival, compared to GNA patients.


Assuntos
Antineoplásicos/farmacologia , Carboplatina/farmacologia , Resistencia a Medicamentos Antineoplásicos , Glucose/deficiência , Glicólise/efeitos dos fármacos , Neoplasias Epiteliais e Glandulares/tratamento farmacológico , Neoplasias Ovarianas/tratamento farmacológico , Idoso , Animais , Carcinoma Epitelial do Ovário , Proteínas de Ciclo Celular/genética , Proteínas de Ciclo Celular/metabolismo , Linhagem Celular Tumoral , Proliferação de Células/efeitos dos fármacos , Intervalo Livre de Doença , Relação Dose-Resposta a Droga , Feminino , Regulação Enzimológica da Expressão Gênica , Humanos , Estimativa de Kaplan-Meier , Camundongos SCID , Pessoa de Meia-Idade , Proteínas Associadas à Resistência a Múltiplos Medicamentos/genética , Proteínas Associadas à Resistência a Múltiplos Medicamentos/metabolismo , Neoplasias Epiteliais e Glandulares/genética , Neoplasias Epiteliais e Glandulares/metabolismo , Neoplasias Epiteliais e Glandulares/patologia , Neoplasias Ovarianas/genética , Neoplasias Ovarianas/metabolismo , Neoplasias Ovarianas/patologia , Fenótipo , Fatores de Tempo , Ensaios Antitumorais Modelo de Xenoenxerto
7.
Pediatr Blood Cancer ; 64(6)2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-27957799

RESUMO

OBJECTIVES: The aim of this retrospective international analysis was to evaluate the role of risk factors in pediatric patients with adrenocortical carcinoma (ACC) observed in European countries (2000-2013) in an attempt to identify factors associated with poor prognosis. PROCEDURES: Data were retrieved from databases of Germany, France, Poland, and Italy, which form the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT). Patients were less than 18 years old, with at least one of the following tumor-related risk factors: metastases, volume more than 200 cm3 , Cushing syndrome, vascular or regional lymph node invasion, initial biopsy, or incomplete excision. Role of patients' age was also evaluated. RESULTS: Eighty-two patients were evaluated: 62 with localized disease and 20 with metastases. The 3-year progression-free survival (PFS) and overall survival (OS) were 39% and 55% for the whole population, respectively, and 51% and 73% for localized diseases, respectively. Concerning the whole population, PFS and OS were influenced by distant metastases, tumor volume, lymph node involvement, age, and presence of two or more risk factors. Factors significant only at OS were vascular involvement and incomplete surgery. At multivariable analysis, the main factors at PFS were volume more than 200 cm3 (hazard ratio [HR]: 2.6, 95% confidence interval [CI]: 1.18-5.70) and presence of distant metastases (HR: 8.26, 95% CI: 3.49-19.51). The OS was significantly influenced by the presence of metastases (P < 0.0001). Concerning patients with localized tumors, the only significant prognostic factor was volume more than 200 cm3 with a HR of 4.38 (95% CI: 1.60-12.00) for PFS and of 3.68 (95% CI: 1.02-13.30) for OS. CONCLUSIONS: Distant metastases and large tumor volume were the main unfavorable prognostic factors. Presence of two or more factors related to ACC was associated with an aggressive behavior of disease.


Assuntos
Adenocarcinoma , Adenocarcinoma/mortalidade , Adenocarcinoma/patologia , Adenocarcinoma/cirurgia , Adolescente , Criança , Pré-Escolar , Intervalo Livre de Doença , Europa (Continente)/epidemiologia , Feminino , Humanos , Metástase Linfática , Masculino , Fatores de Risco , Taxa de Sobrevida
8.
Pediatr Blood Cancer ; 64(6)2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-27882658

RESUMO

BACKGROUND: Adolescents with cancer are enrolled in clinical trials at far lower rates than children. This report compares the number of adolescents (15-19-year-olds) and children (0-14-year-olds) enrolled in the protocols of the European pediatric Soft tissue sarcoma Study Group (EpSSG) with the number of cases expected to occur. METHODS: The observed-to-expected (O/E) ratio was detected in the EpSSG countries contributing most of the cases, that is, Italy, France, Spain, the Netherlands, United Kingdom, and Ireland. The observed cases included patients enrolled in any of the EpSSG protocols from October 2008 to October 2015, when all EpSSG protocols were open in these countries. The number of expected cases was calculated from the incidence rates estimated throughout the RARECAREnet database in the countries' population-based cancer registries. RESULTS: In the countries considered, 2,118 cases aged 0-19 years were enrolled in the EpSSG trials from 2008 to 2015: 82.8% were children and 17.2% were adolescents. The O/E ratio was 0.30 among patients 15-19 years old, as opposed to 0.64 for those 0-14 years old. The O/E ratio differed for the different subtypes: in adolescents, it was 0.64 and 0.18 for rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma soft tissue sarcomas (NRSTS), respectively; in children, it was 0.77 and 0.50, respectively. The O/E ratios differed across the countries considered. CONCLUSIONS: Adolescents were less well represented than children on the EpSSG protocols, with better enrolment for RMS than for NRSTS for all age groups.


Assuntos
Acesso aos Serviços de Saúde , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/terapia , Adolescente , Adulto , Fatores Etários , Criança , Pré-Escolar , Ensaios Clínicos como Assunto , Europa (Continente)/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos
9.
Eur J Cancer ; 60: 69-82, 2016 06.
Artigo em Inglês | MEDLINE | ID: mdl-27082136

RESUMO

BACKGROUND: Extracranial malignant rhabdoid tumours (MRT) are rare lethal childhood cancers that often occur in infants and have a characteristic genetic mutation in the SMARCB1 gene. The European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) conducted a multinational prospective study of registered cases of extracranial MRT to test an intensive multimodal approach of treatment for children with newly diagnosed extracranial MRT. METHODS: Between December 2005 and June 2014, we prospectively registered 100 patients from 12 countries with a diagnosis of MRT tumour at an extracranial site on the EpSSG Non-Rhabdomyosarcoma Soft Tissue Sarcoma 2005 Study (NRSTS 2005). They were all treated on a standard multimodal protocol of surgery, radiotherapy, and chemotherapy over 30 weeks as follows: vincristine, cyclophosphamide, and doxorubicin (VDCy) at weeks 1, 10, 13, 22, and 28; vincristine was also given alone on weeks 2, 3, 11, 12, 14, 15, 23, 24, 29, and 30. Cyclophosphamide, carboplatin, and etoposide (Cy*CE) was given at weeks 4, 7, 16, 19, and 25. Radiotherapy was recommended for all primary tumour sites and all sites of metastatic disease. RESULTS: Forty-three patients completed the protocol treatment. Median follow-up for alive patients of the complete cohort was 44.6 months (range 11.5-84.6). For the whole cohort, the 3-year event-free survival (EFS) was 32.3% (95% confidence interval [CI] 23.2-41.6%) with a 3-year overall survival (OS) of 38.4% (95% CI 28.8-47.9%). For localised disease, the 4-year EFS was 39.3% (95% CI 28.2-50.1%) with a 4-year OS of 40.1% (95% CI 28.4-51.5%). For metastatic disease, the 2-year EFS was 8.7% (95% CI 1.5-24.2%) with a 2-year OS of 13.0% (95% CI 3.3-29.7%). Multivariable analysis disclosed that all patients ≤1 year of age were associated with at higher risk of death (hazard ratio [HR]: 2.6; 95% CI 1.0-6.8; p-value = 0.0094). Risk of death was also related with gender in metastatic patients (HR for males: 2.9, 95% CI 1.0-8.0; p-value = 0.0077). CONCLUSIONS: The EpSSG NRSTS 2005 protocol of intensive therapy can be delivered to extracranial MRT patients, with a possible improvement in outcome. The outcome, however, remains poor for patients who progress or with metastatic disease.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Tumor Rabdoide/terapia , Sarcoma/terapia , Quimiorradioterapia Adjuvante , Criança , Pré-Escolar , Ciclofosfamida/administração & dosagem , Intervalo Livre de Doença , Doxorrubicina/administração & dosagem , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estadiamento de Neoplasias , Estudos Prospectivos , Tumor Rabdoide/mortalidade , Tumor Rabdoide/patologia , Sarcoma/mortalidade , Sarcoma/patologia , Vincristina/administração & dosagem
10.
Eur J Cancer ; 57: 1-9, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26849118

RESUMO

BACKGROUND: Infantile fibrosarcoma (IFS) is a very rare disease occurring in young infants characterised by a high local aggressiveness but overall with a favourable survival. To try to reduce the total burden of therapy, the European pediatric Soft tissue sarcoma Study Group has developed conservative therapeutic recommendations according to initial resectability. MATERIAL AND METHODS: Between 2005 and 2012, children with localised IFS were prospectively registered. Initial surgery was suggested only if possible without mutilation. Patients with initial complete (IRS-group I/R0) or microscopic incomplete (group II/R1) resection had no further therapy. Patients with initial inoperable tumour (group III/R2) received first-line vincristine-actinomycin-D chemotherapy (VA). Delayed conservative surgery was planned after tumour reduction. Aggressive local therapy (mutilating surgery or external radiotherapy) was discouraged. RESULTS: A total of 50 infants (median age 1.4 months), were included in the study. ETV6-NTRK3 transcript was present in 87.2% of patients where investigation was performed. According to initial surgery, 11 patients were classified as group I, 8 as group II and 31 as group III. VA chemotherapy was first delivered to 25 children with IRS-III/R2 and one with IRS-II/R1 disease. Response rate to VA was 68.0%. Mutilating surgery was only performed in three cases. After a median follow-up of 4.7 years (range 1.9-9.0), 3-year event-free survival and overall survival were respectively 84.0% (95% confidence interval [CI] 70.5-91.7) and 94.0% (95% CI 82.5-98.0). CONCLUSIONS: Conservative therapy is possible in IFS as only three children required mutilating surgery, and alkylating or anthracycline based chemotherapy was avoided in 71.0% of patients needing chemotherapy. VA regimen should be first line therapy in order to reduce long term effects.


Assuntos
Antineoplásicos/uso terapêutico , Fibrossarcoma/tratamento farmacológico , Neoplasias de Tecidos Moles/tratamento farmacológico , Quimioterapia Adjuvante/métodos , Terapia Combinada/métodos , Efeitos Psicossociais da Doença , Dactinomicina/administração & dosagem , Intervalo Livre de Doença , Estudos de Viabilidade , Feminino , Fibrossarcoma/patologia , Fibrossarcoma/cirurgia , Seguimentos , Humanos , Lactente , Metástase Linfática , Masculino , Estadiamento de Neoplasias , Estudos Prospectivos , Reoperação , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Resultado do Tratamento , Vincristina/administração & dosagem , Conduta Expectante
11.
J Pediatr Surg ; 49(9): 1367-71, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25148739

RESUMO

AIM: Adrenocortical tumors are very rare in children. The distinction between adenoma and carcinoma is complex because of their clinical/histological characteristics. The analysis of the cases registered in two consecutive Italian Studies is described, in order to provide additional insight into their nature and possibly identify benign and malignant lesions. MATERIALS AND METHODS: The analysis includes patients registered from?? 1.1982 to 6.2011 into two consecutive Italian protocols. RESULTS: Fifty-eight children (age 2-210months) were evaluated. Endocrine manifestations were the most frequent symptoms. Stage distribution at diagnosis was: ST I 35, ST II 17, ST III 1, ST IV 5. Treatment consisted in mitotane for ST II, mitotane+chemotherapy for ST III/IV. Forty-four patients are alive without evidence of disease, 1 is alive with disease, 12 died of disease and 1 because of cardiomyopathy. The Wienecke score system was applied in 24 patients with good significance. A p53 mutation was found in 7 cases, and it was diagnostic for Li-Fraumeni syndrome in 2 benign tumors. CONCLUSIONS: The results highlight the importance of a complete excision to obtain the cure of patients. The efficacy of chemotherapy is controversial, however it was able to control the disease in 4 patients in ST II. The value of the Wienecke score system in predicting patients' outcome was confirmed. p53 mutation was more frequent in malignant tumors and represented the sentinel of the Li-Fraumeni syndrome.


Assuntos
Neoplasias do Córtex Suprarrenal/genética , Neoplasias do Córtex Suprarrenal/patologia , Genes p53/genética , Neoplasias do Córtex Suprarrenal/mortalidade , Neoplasias do Córtex Suprarrenal/cirurgia , Antineoplásicos/uso terapêutico , Quimioterapia Adjuvante , Criança , Pré-Escolar , Feminino , Humanos , Imuno-Histoquímica , Itália , Masculino , Mutação , Invasividade Neoplásica , Sistema de Registros , Estudos Retrospectivos , Análise de Sequência de DNA , Análise de Sobrevida
12.
Eur J Cancer ; 48(9): 1370-5, 2012 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-22322070

RESUMO

BACKGROUND: European protocols for paediatric synovial sarcoma (SS) require that all children routinely undergo chest computed tomography (CT) scanning and bone scanning as initial staging procedures. This study aims to determine the rate of initial metastases in paediatric SS based on specific clinical characteristics, thereby investigating whether these diagnostic procedures are really necessary in all patients. METHODS: Data on 258 previously-untreated SS patients <21 years old were pooled from the databases of different European paediatric groups (study period 1988-2005) for this analysis, and the associations between patients' characteristics and any presence of metastasis were estimated. RESULTS: Fifteen cases (5.8%) had distant metastases at diagnosis (86% pulmonary). The presence of metastases was unassociated with patients' gender or age, tumour grade or site, but it was influenced by T-status, and especially primary tumour size: the risk of metastases was 32 times higher in cases of tumour >5 cm than for tumours ≤ 5 cm. CONCLUSIONS: Our findings suggest that tumour diameter can be used as a variable for identifying patients at greater risk of metastases and warranting more accurate radiological investigations. Chest CT scanning may improve the accuracy of pulmonary staging over X-ray, but requires different ionising radiation exposures that might have carcinogenic potential: it can be omitted for patients with tumours ≤ 5 cm. Given the very low risk of bone metastases, bone scans may be recommended only in cases with evidence of lung metastases.


Assuntos
Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patologia , Adolescente , Neoplasias Ósseas/patologia , Neoplasias Ósseas/secundário , Criança , Feminino , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/secundário , Masculino , Metástase Neoplásica , Estadiamento de Neoplasias/métodos , Estudos Prospectivos , Sistema de Registros , Tomografia Computadorizada por Raios X/métodos
13.
Epidemiol Prev ; 35(3-4): 200-6, 2011 May-Aug.
Artigo em Italiano | MEDLINE | ID: mdl-21914916

RESUMO

INTRODUCTION: Official statistics on mortality represent one of the most important indicators on the health status of a population. Cancer mortality time trends show that southern Italy is progressively losing its lower mortality gap as compared to the North of the country. The mortality contribution of this pattern at a fine geographical scale (provinces) has not been extensively studied in southern Italy. OBJECTIVE: To provide a cancer mortality profile in the 23 provinces of South Italy. DESIGN: Descriptive geographical study at population level. SETTING: Cancer mortality analysis by main causes of death and gender, conducted between 1999 and 2003. We computed age-standardized rates (reference World population) (ASR) as well as standardized mortality ratios (South Italy as reference population) (SMR) to compare mortality between geographical areas. MAIN OUTCOME MEASURES: Mortality from all cancer causes and from major neoplasms. RESULTS: In southern Italy, ASRs for all cancer causes (benign and malignant) were 149.3/105 in males and 81.0/105 in females. In both genders, these rates were lower than rates in Italy as a whole (-8% in males and -9% in females). Cancer mortality ASRs for liver, prostate, urinary organs, melanoma and skin, and neoplasms of lymphatic and haematopoietic tissues displayed weak, or barely any, differences between the South and Italy as a whole. Statistically significant (p<0.01) mortality excesses were observed in 7 out of 23 examined provinces, with highest frequency excesses in Naples and Caserta provinces. In males liver cancer mortality showed a SMR excess of 30- 50% in 4/23 provinces. Female breast cancer mortality displayed a 10% excess in 3/23 provinces. CONCLUSIONS: Although this analysis highlighted a lower mortality in southern Italy, as compared to Italy as a whole, seven provinces showed excesses mainly for liver and breast cancers. Current knowledge on cancer etiology explains the vast majority of such observed excesses. Further analyses will help in designing and monitoring cancer prevention and early diagnosis interventions also in South Italy.


Assuntos
Neoplasias/mortalidade , Distribuição por Idade , Feminino , Humanos , Itália/epidemiologia , Masculino , Distribuição por Sexo
14.
Pediatr Blood Cancer ; 57(7): 1261-5, 2011 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-21826783

RESUMO

PURPOSE: Patients with non-metastatic rhabdomyosarcoma (RMS) have a cure rate of 50-90%, but up to one-third of them experience mostly a local recurrence. Second-line treatment is not standardized as for newly diagnosed tumors. We evaluated the role of surgery on local relapses in a series of patients with RMS. METHODS: This retrospective analysis involves 70 patients enrolled in two consecutives Italian Studies, RMS88 and RMS96, who presented local recurrence. After relapse, 40/70 underwent a surgical excision (Surgery Group, SG), that was demolitive in 10/40; 24/40 had radiotherapy, 16/40 did not receive radiotherapy or data are not known. Thirty patients out of 70 did not receive any surgical treatment (No-Surgery Group, NSG), and 20/30 received radiotherapy. RESULTS: Overall survival (OS) after local relapse was 41.6% (mean follow-up 59 months, range 1-226). OS of SG patients was 54% versus 24.7% of the NSG patients (P = 0.0117). Furthermore, OS among the SG was 61.4% with and 41.8% without radiotherapy, and 37.1% with and 0% without radiotherapy among the NSG (P < 0.0001). One patient developed a second local relapse after excision without radiotherapy for the first one, and was cured with further treatment. Demolitive surgery did not improve survival compared to conservative surgery (40% vs. 58.4%, P = 0.1462). CONCLUSION: The treatment of recurrent RMS represents a challenge. In our experience, patients with local relapse had a poor prognosis. SG patients had a better outcome than NSG patients and those treated with resection plus radiotherapy had the best outcome; patients who did not receive any local treatment had an unfavorable outcome.


Assuntos
Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/cirurgia , Rabdomiossarcoma/mortalidade , Rabdomiossarcoma/cirurgia , Adolescente , Criança , Feminino , História Medieval , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Rabdomiossarcoma/patologia , Adulto Jovem
15.
Clin Infect Dis ; 51(9): 1099-101, 2010 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-20879866

RESUMO

During the period 1999­2006, non­AIDS-defining cancers accounted for 7.4% of deaths among Italian people with AIDS. The risk of death was 6.6-fold higher than in the general population, being particularly elevated for virus-related cancers. The study findings highlighted the importance of monitoring the cancer burden on mortality for people with AIDS.


Assuntos
Síndrome de Imunodeficiência Adquirida/complicações , Neoplasias/epidemiologia , Neoplasias/mortalidade , Humanos , Itália/epidemiologia , Medição de Risco
16.
AIDS Res Ther ; 7: 11, 2010 May 24.
Artigo em Inglês | MEDLINE | ID: mdl-20497520

RESUMO

After the introduction of highly active antiretroviral therapies (HAART), an increased incidence of insulin resistance, diabetes mellitus (DM), and cardiovascular diseases has been described. The impact of such conditions on mortality in the post-HAART era has been also assessed in various modes in the literature. In this paper, we report on the death risks for DM, myocardial infarction, and chronic ischemic heart diseases that were investigated among 9662 Italian AIDS cases diagnosed between 1999 and 2005. Death certificates reporting DM, myocardial infarction, and chronic ischemic heart diseases were reviewed to identify the underlying cause of death, and to compare the observed numbers of deaths with the expected ones from the sex- and age-matched, general population of Italy. Person-years at risk of death were computed from date of AIDS diagnosis up to date of death or to December 31, 2006. Standardized mortality ratios (SMR) and their 95% confidence intervals (CI) were computed. DM and cardiovascular diseases were the cause of death for 43 out of 3101 deceased AIDS cases (i.e., 1.4% of all deaths). In comparison with the general population, the risks of death were 6.4-fold higher for DM (95% CI:3.5-10.8), 2.3-fold higher for myocardial infarction (95% CI:1.4-3.7) and 3.0 for chronic ischemic heart diseases (95% CI: 1.5-5.2).

17.
Cancer Epidemiol ; 34(3): 257-61, 2010 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-20413362

RESUMO

BACKGROUND: Kaposi sarcoma (KS) and non-Hodgkin lymphoma (NHL) have strongly diminished in the HAART era, but their impact on life expectancy of people with AIDS (PWA) needs to be monitored. We aimed at quantifying the burden of KS and NHL on mortality of PWA in the HAART period in Italy. METHODS: Death certificates of 3209 PWA diagnosed in 1999-2006 who died as of December 2006 were reviewed to identify those deaths in which KS or NHL was the underlying cause. Standardized mortality ratios (SMR) were computed. RESULTS: KS or NHL appeared in 4.3% and 14.6% death certificates, respectively; they were the underlying cause of death in 3.1% and 13.4% of cases. SMR were 8698-fold higher for KS and 349-fold higher for NHL, and tended to decline over the study period. CONCLUSION: KS and NHL caused about 16% of deaths of PWA in the HAART era, with 100-fold higher risks of death compared to the Italian general population also in recent years. Clinicians and public health officials should be aware of the persisting negative impact of these cancers on life expectancy of PWA.


Assuntos
Síndrome de Imunodeficiência Adquirida/mortalidade , Terapia Antirretroviral de Alta Atividade , Linfoma não Hodgkin/mortalidade , Sarcoma de Kaposi/mortalidade , Síndrome de Imunodeficiência Adquirida/complicações , Síndrome de Imunodeficiência Adquirida/tratamento farmacológico , Adulto , Feminino , Humanos , Linfoma não Hodgkin/complicações , Masculino , Pessoa de Meia-Idade , Vigilância da População , Sarcoma de Kaposi/complicações
18.
Int J Cancer ; 127(6): 1437-45, 2010 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-20049835

RESUMO

People with HIV/AIDS (PWHA) have increased risk of some cancers. The introduction of highly active antiretroviral therapies (HAART) has improved their life expectancy, exposing them to the combined consequences of aging and of a prolonged exposure to cancer risk factors. The aim of this study was to estimate incidence rates (IR) in PWHA in Italy, before and after the introduction of HAART, after adjusting for sex and age through direct standardization. An anonymous record linkage between Italian AIDS Registry (21,951 cases) and Cancer Registries (17.3 million, 30% of Italian population) was performed. In PWHA, crude IR, sex- and age-standardized IR and age-specific IR were estimated. The standardized IR for Kaposi sarcoma and non-Hodgkin lymphoma greatly declined in the HAART period. Although the crude IR for all non-AIDS-defining cancers increased in the HAART period, standardized IR did not significantly differ in the 2 periods (352 and 379/100,000, respectively). Increases were seen only for cancer of the liver (IR ratio = 4.6, 95% CI: 1.3-17.0) and lung (IR ratio = 1.8, 95% CI: 1.0-3.2). Age-specific IRs for liver and lung cancers, however, largely overlapped in the 2 periods pointing to the strong influence of the shift in the age distribution of PWHA on the observed upward trends. In conclusion, standardized IRs for non-AIDS-defining cancers have not risen in the HAART period, even if crude IRs of these cancers increased. This scenario calls, however, for the intensification of cancer-prevention strategies, notably smoking cessation and screening programs, in middle-aged HIV-patients.


Assuntos
Síndrome de Imunodeficiência Adquirida/complicações , Neoplasias/epidemiologia , Humanos , Incidência , Itália/epidemiologia , Neoplasias/complicações
19.
Support Care Cancer ; 18(9): 1191-7, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19784676

RESUMO

GOALS OF WORK: Fatigue is the most distressing untreated symptom for many cancer patients, and its measurement is of great topical interest. The aim of the present study was to assess psychometric properties of Revised Piper Fatigue Scale (PFS-r) in Italian cancer patients. PATIENTS AND METHODS: From January to June 2007, 115 histologically confirmed cancer inpatients (age >or=18 years; Eastern Cooperative Oncology Group [ECOG] performance status or=0.97) for all subscales. Exploratory factor analysis revealed three dimensions instead of four in the US questionnaire; 68.2% of the common variance was explained. Internal consistency was satisfactory (Cronbach's alpha >0.80) as was the test-retest reliability. Good correlations between PFS-r subscale and POMS subscales confirmed criterion validity. CONCLUSIONS: The psychometric properties of the Italian version of PFS-r, as evaluated in cancer patients ongoing chemotherapy, were satisfactory. We suggest the possible implementation of the Italian PFS-r in the assessment of fatigue particularly when it has been more fully validated on a wider range of cancer patients.


Assuntos
Fadiga , Neoplasias/complicações , Inquéritos e Questionários , Análise Fatorial , Feminino , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Neoplasias/fisiopatologia , Psicometria
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