RESUMO
The outcome of high-risk (HR) acute lymphoblastic leukemia patients enrolled in the AIEOP-BFM ALL 2000 study in Italy is described. HR criteria were minimal residual disease (MRD) levels ≥10(-3) at day 78 (MRD-HR), no complete remission (CR) at day 33, t(4;11) translocation, and prednisone poor response (PPR). Treatment (2 years) included protocol I, 3 polychemotherapy blocks, delayed intensification (protocol IIx2 or IIIx3), cranial radiotherapy, and maintenance. A total of 312 HR patients had a 5-year event-free survival (EFS) of 58.9% (standard error [SE] = 2.8) and an overall survival of 68.9% (SE = 2.6). In hierarchical order, EFS was 45.9% (4.4) in 132 MRD-HR patients, 41.2% (11.9) in 17 patients with no CR at day 33, 36.4% (14.5) in 11 patients with t(4;11), and 74.0% (3.6) in 152 HR patients only for PPR. No statistically significant difference was found for disease-free survival in patients with very HR features [MRD-HR, no CR at day 33, t(4;11) translocation], given hematopoietic stem cell transplantation (HSCT) (n = 66) or chemotherapy only (n = 88), after adjusting for waiting time to HSCT (5.7 months). Patients at HR only for PPR have a favorable outcome. MRD-HR is associated with poor outcome despite intensive treatment and/or HSCT and may qualify for innovative therapies. The study was registered at www.clinicaltrials.gov as #NCT00613457.
Assuntos
Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Terapia Combinada , Feminino , Transplante de Células-Tronco Hematopoéticas , Humanos , Lactente , Masculino , Neoplasia Residual , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Radioterapia , Indução de Remissão , Resultado do TratamentoRESUMO
BACKGROUND: BK polyomavirus infects most of the general population. However, its clinical manifestations are almost exclusively seen in immunocompromised patients, particularly in kidney and hematopoietic stem cell transplantation recipients. CASE PRESENTATION: A 15-y-old female suffering from common B-cell acute lymphoblastic leukaemia underwent hematopoietic stem cell transplantation. The patient had reactivation of BKPyV infection and developed an haemorrhagic cystitis. Three months after transplant, BKPyV viremia and viruria increased and she developed a severe nephropathy associated to a polyclonal gammopathy with high levels of isolated IgM. CONCLUSION: This case report describes a rare and unexpected polyclonal gammopathy developed during a polyomavirus-associated nephropathy confirmed by immunohistochemical and laboratory analyses.
Assuntos
Anticorpos Antivirais/imunologia , Vírus BK/fisiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Nefropatias/etiologia , Leucemia de Células B/terapia , Plasmócitos/citologia , Infecções por Polyomavirus/imunologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Adolescente , Vírus BK/imunologia , Feminino , Humanos , Nefropatias/imunologia , Nefropatias/virologia , Leucemia de Células B/complicações , Plasmócitos/imunologia , Infecções por Polyomavirus/etiologia , Infecções por Polyomavirus/virologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicaçõesRESUMO
BACKGROUND: Teratomas demonstrate a benign clinical behavior, however they may recur with malignant components or as teratoma, and in a small group of patients prognosis could be fatal. After the first Italian study, we collected cases of teratoma, alongside the protocol for malignant germ cell tumors. PROCEDURE: Patients with teratoma were collected from 2004 to 2014. Teratomas were classified according to the WHO classifications, as mature and immature. Patients with pathological aFP and/or bHCG, and those with a malignant germ cell component were not included. RESULTS: The study enrolled 219 patients (150 mature, 69 immature teratomas) with a median age at diagnosis of 42 months. The primary sites involved were: 118 gonadal and 101 extragonadal teratomas. Two females with ovarian teratoma had a positive family history. Complete and incomplete surgeries were performed in 85% and 9% of cases. Seventeen events occurred: six females had a second metachronous tumor (5 contralateral ovarian teratoma, 1 adrenal neuroblastoma) and 11 teratomas relapsed/progressed (3 mature, 8 immature teratomas). Two patients died, one of progressive immature teratoma and one of surgical complications. At a median follow up of 68 months, the event-free, relapse-free, and overall survival rates were 90.6%, 94.3%, 98.6%, respectively. CONCLUSIONS: Teratomas show a good prognosis, especially the mature ones: surgery and follow-up remain the standard approach. Incomplete surgery in immature teratoma is the group at greatest risk of relapse. Bilateral ovarian tumors are a possibility, and the rare family predisposition to ovarian mature teratoma warrants further analyses.
Assuntos
Recidiva Local de Neoplasia/epidemiologia , Segunda Neoplasia Primária/epidemiologia , Neuroblastoma/epidemiologia , Neoplasias Ovarianas/epidemiologia , Teratoma/epidemiologia , Neoplasias Testiculares/epidemiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Incidência , Lactente , Recém-Nascido , Itália/epidemiologia , Masculino , Gradação de Tumores , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Segunda Neoplasia Primária/mortalidade , Segunda Neoplasia Primária/patologia , Neuroblastoma/mortalidade , Neuroblastoma/patologia , Neoplasias Ovarianas/mortalidade , Neoplasias Ovarianas/patologia , Prognóstico , Estudos Prospectivos , Taxa de Sobrevida , Teratoma/mortalidade , Teratoma/patologia , Neoplasias Testiculares/mortalidade , Neoplasias Testiculares/patologia , Adulto JovemRESUMO
Teno Torque virus, member of the family of Anelloviridae, has been associated with many autoimmune diseases such as idiopathic hepatitis, systemic lupus erythematosus, and multiple sclerosis. Its viral load tends to be higher in the bone marrow and in tissues with high turnover rate. We report here a case of an 11-month-old infant affected by acute myeloid leukemia who underwent hematopoietic stem cell transplantation, and after 6 months had autoimmune hepatitis and atopic dermatitis. Extremely high-cytokine IP-10 and eotaxin levels were found in her sera, and serological tests and RT-PCR for viruses showed positive results exclusively for Teno Torque virus.
Assuntos
Doenças Autoimunes/etiologia , Autoimunidade/imunologia , Infecções por Vírus de DNA/virologia , Dermatite Atópica/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Hepatite/etiologia , Leucemia Mieloide Aguda/imunologia , Torque teno virus/patogenicidade , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/patologia , Infecções por Vírus de DNA/imunologia , Infecções por Vírus de DNA/patologia , DNA Viral/genética , Dermatite Atópica/tratamento farmacológico , Dermatite Atópica/patologia , Feminino , Hepatite/tratamento farmacológico , Hepatite/patologia , Humanos , Lactente , Leucemia Mieloide Aguda/terapia , Leucemia Mieloide Aguda/virologia , Prognóstico , Torque teno virus/genética , Torque teno virus/isolamento & purificação , Carga ViralRESUMO
Hurler syndrome type 1 (MPS-1) is an autosomal recessive lysosomal disorder due to the deficiency of the enzyme alpha-L-iduronidase which is necessary for the degradation of dermatan and heparan sulfate. It is characterized by deposit of glycosaminoglycans in tissues, progressive multisystem dysfunction, and early death. HSCT for children with MPS-I is effective, resulting in increased life expectancy and improvement of clinical parameters. The spinal MRI performed on a female 10 yr old undergoing HSCT at the age of 18 months and receiving ERT revealed a considerable decrease in soft tissue around the tip of odontoid causing a significant reduction in spinal cord compression. In light of this result, we suppose that combined ERT and HSCT are successful in Hurler I disease.
Assuntos
Transplante de Células-Tronco Hematopoéticas/métodos , Iduronidase/uso terapêutico , Mucopolissacaridose I/terapia , Compressão da Medula Espinal/terapia , Transplante de Medula Óssea , Criança , Terapia de Reposição de Enzimas/métodos , Feminino , Glicosaminoglicanos/urina , Humanos , Iduronidase/urina , Imageamento por Ressonância Magnética , Processo Odontoide/fisiopatologia , Resultado do TratamentoRESUMO
OBJECTIVES: The aim of this study is to evaluate the fertility in young patients affected by Hodgkin and non-Hodgkin lymphoma, before and after chemotherapy and/or radiotherapy. We conducted a retrospective study to analyse how treatment affects male fertility and a perspective study to assess pre-treatment sperm quality. MATERIALS AND METHODS: 28 patients, treated in our center or referred to our Medically Assisted Procreation Center, from 2002 to 2011, were selected for the retrospective study and asked if interested in their fertility assessment. Semen samples were taken from 11 patients (mean age 31.55: range 20-45); other possible causes of impaired fertility were excluded. We analyzed pretreatment semen samples of 61 patients (mean age 29.08 +/- 9.5) affected by leukaemia or lymphoma that were selected for the perspective study and referred to the Sperm Bank of Pordenone. All semen samples were analysed accordingly to 1999 World Health Organization guidelines. RESULTS: In the retrospective study all semen samples of the 11 patients selected were altered. Six patients treated with high dose alkylating agents and abdominal/pelvic radiotherapy were found azoospermic, 3 with severe oligoasthenozoospermic, 1 oligoteratozoospermic and 1 asthenozoospermic. In the perspective study pretreatment semen quality was poor in most of the samples of the 61 patients selected. Normozoospermia was observed in 14% of patients affected by Hodgkin lymphoma and in 25% affected by non-Hodgkin lymphoma. CONCLUSION: Chemotherapy, radiotherapy or their combination are followed by a temporary but sometimes irreversible reduction of fertility potential. Pre-treatment semen quality is acceptable to proceed with cyopreservation techniques. Sperm cryopreservation should be offered to all post puberal male patients who have not yet conceived before treatment with gonado-toxic agents.
Assuntos
Doença de Hodgkin/complicações , Doença de Hodgkin/terapia , Infertilidade Masculina/etiologia , Linfoma não Hodgkin/complicações , Linfoma não Hodgkin/terapia , Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Teratoma with a malignant somatic component (TMSC) is rare but described in adults, whereas information on pediatric presentation is sparse. PROCEDURE: The Associazione Italiana Ematologia Oncologia Pediatrica identified 14 cases of TMSC. Clinical files and pathology specimens were reviewed. RESULTS: The series (9 female, 5 male) showed the following disease: testis (2), sacrococcygeal (3), ovary (3), retroperitoneum (3), mediastinum (2), and foot soft tissue (1). Distribution of the somatic component was: carcinoma (4), pancreatic neuroendocrine tumor (1), neuroblastoma (3), rhabdomyosarcoma (3), rhabdomyosarcoma plus liposarcoma, chondrosarcoma, neurogenic sarcoma (1), chondrosarcoma plus neuroectodermal sarcoma (1), malignant peripheral nerve sheath tumor (1). Three patients were in stage I, four in stage II, three in stage III, and four in stage IV. All but one patient underwent surgery and only females showed carcinoma components. Nine patients relapsed or progressed and eight died. Six patients are alive and disease-free. Two patients underwent complete resection and four were treated based on transformed histologies. Relapse-free and overall survival rates were 35.7% and 42.8%, respectively (median follow-up, 31 months). CONCLUSIONS: Prognosis for germ cell tumors (GCTs) containing MSC is worse than that for GCTs. The pediatric disease appears to be more heterogeneous in tumor site distribution and MSC histology than in adults. Our series suggests no effects of age, histology, or gender on outcome. Surgery has an essential role in localized disease, with complete resection highly desirable. Chemotherapy optimized for histology should include reagents directed to the somatic malignancy, if chemosensitive. Malignant GCT warrants GCT-directed chemotherapy.
Assuntos
Teratoma/patologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Itália , Masculino , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Teratoma/mortalidade , Teratoma/cirurgia , Resultado do TratamentoRESUMO
BACKGROUND: Glucocorticoids (GCs) play a fundamental role in the treatment of pediatric acute lymphoblastic leukemia (ALL), but therapy with these agents often results in a number of severe side effects. The aim of our study was to evaluate the association between polymorphisms of genes encoding for proteins involved in the pharmacokinetics/pharmacodynamics of these drugs and the occurrence of side effects, in particular infections, in a small population of ALL children. PROCEDURE: Common polymorphisms of NR3C1, ABCB1, glutathione-S-transferase (GST)-M1, GST-P1, GST-T1, and IL-10 genes were analyzed in 36 pediatric patients with ALL, treated according to the AIEOP-BMF ALL 2000 study protocol. Toxicities occurring during the induction and reinduction periods were assessed and their association with genotypes was evaluated. RESULTS: In univariate analysis, the risk of severe infections was increased in subjects with the GST-M1 null genotype, while patients with the GST-M1 normal genotype had significantly more moderate degree infections. The results were confirmed by multivariate analysis. Selection from the reference models of independent variables based on Akaike Information Criteria (AIC) scores maintained the GST-M1 genotype variable in the model to predict severe infections, and the ABCB1 C3435T and GST-M1 genotype variables in the model for moderate infections. CONCLUSIONS: GST-M1 genotype may influence the severity of infections in ALL children during GC administration.
Assuntos
Glucocorticoides/administração & dosagem , Glutationa Transferase/genética , Polimorfismo Genético , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Dexametasona/administração & dosagem , Feminino , Glucocorticoides/toxicidade , Humanos , Lactente , Infecções/induzido quimicamente , Infecções/genética , Masculino , Metotrexato/administração & dosagem , Farmacogenética , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Prednisona/administração & dosagemRESUMO
OBJECTIVE: to report and analyse time trends in cancer incidence among children (0-14 years of age), adolescents (15-19 years) and young adults (20-24 years) living in the Italian province of Trieste (2003 population, 242,000), between 1972 and 2003. DESIGN: population-based study of descriptive epidemiology. SETTING AND PARTICIPANTS: the new cases of cancer diagnosed to the residents of the province of Trieste below 25 years of age were extracted from the database of the Trieste Cancer Registry (period 1972-1994) and from the database of the Friuli-Venezia Giulia Cancer Registry (period 1995-2003), according to the International Classification of Childhood Cancer (3rd edition). MAIN OUTCOME MEASURES: age-specific and age-standardized (Italian 1981 census population as standard) incidence rates, by diagnostic group, sex and period of diagnosis. Time trend in incidence was analysed by using a Poisson regression model adjusted for calendar year, sex and 5 year age-group, and was expressed as annual percent change (APC) in rates. RESULTS: in the period 1972-2003, the new cases of cancer were 168 in the age-group 0-14 years, 79 in the age-group 15-19 years and 111 in the age-group 20-24 years, while the person-years at risk were respectively: 1,050,027; 431,673; 496,450. The APC in the incidence of all cancers combined was 2.3% (IC 95% 0.6%-3.9%) in children, 4.4% (IC 95% 1.8%-7.1%) in adolescents and 5.1% (IC 95% 2.8%-7.5%) in young adults. Hodgkin lymphomas (APC =12.7%; IC 95% 2.6%-23.7%; 7 cases) in the age-group 0-14 years, skin melanomas and carcinomas (APC =8.2%; IC 95% 4.5%-12.0%; 49 cases) and central nervous system tumours (APC = 6.4%; IC 95% 1.5%-11.5%; 25 cases) in the age-group 15-24 years were the malignancies characterised by the highest increase in incidence. CONCLUSION: the increase in incidence rates observed in this study can be only partly explained by the small number of ascertained cases, by an improvement in diagnostic techniques and by more efficient registration. However, few environmental and hereditary factors are consistently associated with cancers affecting young people. Therefore, it is imperative to continue to carry out descriptive and analytical studies with primary prevention as the ultimate aim.
Assuntos
Neoplasias/epidemiologia , Idade de Início , Viés , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Itália/epidemiologia , Modelos Lineares , Masculino , Morbidade/tendências , Neoplasias/diagnóstico , Distribuição de Poisson , Sistema de Registros/estatística & dados numéricos , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: The outcome of patients with non-extremity synovial sarcoma (SS) is generally worse than that of patients with limb tumours. METHODS: The present study analysed a series of 115 consecutive SS patients treated in Italian paediatric protocols (period 1979-2005), mainly focusing on the 30 cases arising from 'axial' sites (16 head-neck, 8 trunk, 4 lung-pleura and 2 retroperitoneum). RESULTS: Initial gross resection was achieved in 40% of axial cases and in 80% of limb SS (p<0.0001). Five-year EFS and overall survival (OS) were, respectively, 43.3% and 55.1% for axial SS, and 69.6% (p=0.0068) and 84.0% (p=0.0004) for extremity SS. Local progression/recurrence was the cause of treatment failure in 75% of relapsing patients axial disease. CONCLUSIONS: Our findings emphasise that children and adolescents with SS originating at non-extremity locations have a worse prognosis than those with limb SS. Tumour site should be considered when defining a risk-adapted treatment strategy for SS.
Assuntos
Sarcoma Sinovial/terapia , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Terapia Combinada/mortalidade , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Itália/epidemiologia , Masculino , Estadiamento de Neoplasias , Neoplasia Residual , Estudos Prospectivos , Sarcoma Sinovial/mortalidade , Sarcoma Sinovial/patologia , Resultado do TratamentoRESUMO
PURPOSE: To identify the prognostic factors, treatment, and outcome of children affected by renal cell carcinoma (RCC). PATIENTS AND METHODS: The series included 41 patients (18 males and 23 females) with a median age of 124 months observed at the 11 Italian Association for Pediatric Hematology and Oncology centers from January 1973 to January 2001. Clinical data, surgical notes, pathologic findings, and summaries of therapy were taken from the charts. RESULTS: Seven (17%) of the 41 patients had a papillary histology, and 34 (82.4%) had nonpapillary histology. Eighteen patients (43.9%) had stage I, one patient (2.4%) had stage II, two patients (4.8%) had stage IIIA, 10 patients (24.3%) had stage IIIB, and nine patients (21.9%) had stage IV disease. One patient had a bilateral involvement at diagnosis. Seven patients experienced disease recurrence. Lung and liver were the most common distant lesions and usually were fatal. In this study, the major factor influencing the prognosis was the stage. Event-free survival at 20 years was 53.5% for all patients. Overall survival at 20 years was 54.9% for all patients. CONCLUSION: RCC is a rare disease in children and adolescents. This neoplasm has a different clinical presentation in children compared with adults but the same outcome. In our experience, patients with localized disease could be cured by nephrectomy alone. Prospective studies in a larger number of patients are needed to confirm radiation therapy and biologic response modifiers as effective adjunct therapy in RCC stage III. The alternative therapy seems warranted in patients with advanced disease.
Assuntos
Carcinoma de Células Renais/patologia , Neoplasias Renais/patologia , Recidiva Local de Neoplasia , Nefrectomia , Adolescente , Carcinoma de Células Renais/radioterapia , Carcinoma de Células Renais/cirurgia , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Neoplasias Renais/radioterapia , Neoplasias Renais/cirurgia , Masculino , Metástase Neoplásica , Estadiamento de Neoplasias , Prognóstico , Fatores de Risco , Resultado do TratamentoRESUMO
Varicella zoster virus (VZV), a threat for hematopoietic stem cell transplantation (HSCT) recipients, is still one of the most common viral pathogens that affect these patients with a reported incidence ranging between 17% and 50% in the post transplantation period. Valganciclovir (V-GCV), a valine ester pro-drug of GCV orally administrable, has recently shown great activity against CMV infections, but there are no reports of its clinical efficacy against VZV. We here report a case history of a patient with positive serologic test for VZV, who underwent allogeneic HSCT and developed an atypical varicella-like illness. First-line therapy with foscarnet had to be discontinued due rapid development of renal impairment (creatinine: 2.60 mg/dL, urea: 130.6 mg/dL) and therefore was switched to V-GCV. The renal impairment and skin lesions of the patient fully recovered after few days of therapy, even though the patient had complete lymphocyte depletion. This is the first case of a patient with chickenpox-like illness treated successfully with V-GCV.
Assuntos
Injúria Renal Aguda/induzido quimicamente , Transplante de Medula Óssea/efeitos adversos , Ganciclovir/análogos & derivados , Herpes Zoster/tratamento farmacológico , Herpesvirus Humano 3/crescimento & desenvolvimento , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Adolescente , Evolução Fatal , Feminino , Ganciclovir/uso terapêutico , Humanos , Valganciclovir , Carga ViralRESUMO
BACKGROUND AND OBJECTIVES: Osteonecrosis (ON) is a potentially disabling complication of combination chemotherapy including high doses of steroids. The incidence and main risk factors for symptomatic ON have been investigated in a large group of children treated with high-dose steroids, prednisone and dexamethasone for childhood acute lymphoblastic leukemia (ALL). DESIGN AND METHODS: From May 1995 to December 1999, 1421 patients <18 years old, with newly diagnosed non-B ALL, were registered in the AIEOP-ALL 95 study. Their data were reviewed to identify patients who developed symptomatic ON. For those who were positively identified additional data were requested concerning ON-related symptoms, treatment and outcome. RESULTS: Overall, 15 of the 1421 patients developed symptomatic ON (1.1%) in a total of 29 sites. The estimated 5-year cumulative risk for clinically diagnosed ON was 1.6% (SE 0.4). The incidence was significantly higher among females (p=0.01) and older patients, with a peak rate of 7.4% (2.3) among those aged 10 to 17 years (p<0.0001). When the two factors, i.e. age and gender were combined, there was a striking increase in the risk among female patients aged 10 to 17 years. The median time between the diagnosis of ALL and that of ON was 17 months (range 8-45). The hip was the most frequently involved (19/29) site. INTERPRETATION AND CONCLUSIONS: Symptomatic ON occurred in only 1.1% of patients treated with BFM-type, intensive chemotherapy for childhood ALL. Female adolescents appear to be the subset of patients with the highest risk of ON, especially when categorized as having high risk leukemia and thus administered higher cumulative doses of dexamethasone.
Assuntos
Antineoplásicos Hormonais/efeitos adversos , Osteonecrose/induzido quimicamente , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Adolescente , Antineoplásicos Hormonais/uso terapêutico , Criança , Dexametasona/efeitos adversos , Dexametasona/uso terapêutico , Quimioterapia Combinada , Feminino , Humanos , Incidência , Masculino , Osteonecrose/diagnóstico , Osteonecrose/epidemiologia , Osteonecrose/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Prednisona/efeitos adversos , Prednisona/uso terapêutico , Fatores de Risco , Resultado do TratamentoRESUMO
We analysed incidence, survival and mortality from cancer among people aged 15-24 years resident in the province of Trieste, Italy, during 1972-1993, and evaluated the quality of the local diagnostic facilities and of the care provided by local hospital departments. We compared the results with those previously published on childhood cancer. We recorded 118 new cases of cancer (96% microscopically verified) corresponding to a rate, age-standardized to the world population, of 162.3 (standard error SE = 15.0) per million person-years. The diagnostic group with the highest rate was that of carcinomas (54.5; SE = 8.7; 40 cases). The diagnosis was reached at hospitals in the province of Trieste for 107 cases, with a median time of nine days (25th-75th percentile = 5-23) between admission and diagnosis. Among patients with leukaemias, lymphomas and brain tumours, this interval was longer than in children affected by the same neoplasms. One girl with cancer of the uterine cervix refused all treatment. The therapy of the other 117 cases were co-ordinated by 29 different departments of hospitals located in Trieste for 86 cases, in other Italian hospitals for 26 cases and in European hospitals for five cases. On the other hand, out of 123 childhood cancers 107 were co-ordinated by three departments in Trieste, seven by other Italian hospitals and nine by foreign hospitals. The 10-year survival probabilities of children with acute lymphoblastic leukaemia and non-Hodgkin lymphomas were higher than those of patients aged 15-24 years: 66.7% (SE = 9.1) vs 14.3% (SE = 13.2) and 77.8% (SE = 13.9) vs 40.0% (SE = 21.9), respectively.
Assuntos
Neoplasias/epidemiologia , Adolescente , Adulto , Área Programática de Saúde , Feminino , Humanos , Incidência , Itália/epidemiologia , MasculinoRESUMO
BACKGROUND: There has been a noticeable annual increase in the number of children coming to Italy for medical treatment, just like it has happened in the rest of the European Union. In Italy, the assistance to children suffering from cancer is assured by the current network of 54 centres members of the Italian Association of Paediatric Haematology and Oncology (AIEOP), which has kept records of all demographic and clinical data in the database of Mod.1.01 Registry since 1989. METHODS: We used the information stored in the already mentioned database to assess the impact of immigration of foreign children with cancer on centres' activity, with the scope of drawing a map of the assistance to these cases. RESULTS: Out of 14,738 cases recorded by all centres in the period from 1999 to 2008, 92.2% were born and resident in Italy, 4.1% (608) were born abroad and living abroad and 3.7% (538) were born abroad and living in Italy. Foreign children cases have increased over the years from 2.5% in 1999 to. 8.1% in 2008.Most immigrant children came from Europe (65.7%), whereas patients who came from America, Asia and Oceania amounted to 13.2%, 10.1%, 0.2%, respectively. The immigrant survival rate was lower compared to that of children who were born in Italy. This is especially true for acute lymphoblastic leukaemia patients entered an AIEOP protocol, who showed a 10-years survival rate of 71.0% vs. 80.7% (p < 0.001) for immigrants and patients born in Italy, respectively. CONCLUSIONS: Children and adolescents are an increasingly important part of the immigration phenomenon, which occurs in many parts of the world. In Italy the vast majority of children affected by malignancies are treated in AIEOP centres. Since immigrant children are predominantly treated in northern Italy, these centres have developed a special expertise in treating immigrant patients, which is certainly very useful for the entire AIEOP network.
Assuntos
Emigrantes e Imigrantes/estatística & dados numéricos , Neoplasias/etnologia , Adolescente , África/etnologia , Ásia/etnologia , Criança , Pré-Escolar , Bases de Dados Factuais , Etnicidade/estatística & dados numéricos , Europa Oriental/etnologia , União Europeia/estatística & dados numéricos , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Itália/epidemiologia , Masculino , Neoplasias/mortalidade , América do Norte/etnologia , Oceania/etnologia , Prevalência , Estudos Retrospectivos , América do Sul/etnologia , Taxa de SobrevidaRESUMO
Teratoma is the most common germ cell tumour in childhood; mature (MT) and immature teratomas (IT) are benign tumours, but if they recur, they can be in some cases malignant. The aim of this paper is to evaluate Italian patients with MT and IT enrolled from 1991 to 2001, in a prospective multicentric study. One hundred and eighty-three patients, observed in 15 Italian Centers of Paediatric Oncology and three Paediatric Surgical Units were enrolled. Clinical data, treatment and results were all analysed. Initial evaluation and subsequent follow up included clinical examination, tumour markers and imaging procedures. Surgical resection was recommended for all the tumours. Histology was centrally reviewed and IT was classified as grading 1-3. Chemotherapy (CT) with Vinblastine, D: -actinomycin and cyclophosphamide was indicated for extra-testicular IT grade 2 or 3. MT was diagnosed in 127 patients (93 F and 34 M, age 1-192 months, median 24): 58 patients had gonadic tumour (23 testicular, 35 ovaric), 69 extragonadic (45 sacrococcygeal, 11 mediastinic, 7 retroperitoneal, 6 in other sites). A complete resection was performed in 117 patients, a partial resection in eight patients and biopsy in one. IT was diagnosed in 56 patients (34 F, 22 M, age 1-168 months, median 7). The T grading was 1 in 14 cases, 2 in 26, 3 in 16; 28 had gonadic T (17 ovary, 11 testis), 28 extragonadic (sacrococcygeal 19, mediastinic 3, retroperitoneal 2, other sites 4). CT was administered in eight patients; 15/182 patients relapsed (1 in a metastatic site) and in 5/15 the relapse showed malignant histology. Seven MT (5.5%) relapsed (five sacrococcygeal, one retroperitoneal, one mediastinic): surgery at diagnosis had been complete in five and with residual in two; the relapse was malignant in two patients with sacrococcygeal (sc) tumours, who had a complete resection and a partial resection respectively. Eight IT (14.2%) relapsed (four ovary, three sc, one retroperitoneal). The initial surgical resection had been complete in one, with residual in six, and a biopsy had been performed in one. A malignant recurrence occurred in two patients with sc tumours (after partial resection in one and after biopsy + CT in one) and in one patient with ovarian IT after a partial resection. All the patients underwent surgical excision of the recurred mass; CT according to Protocol for Malignant GCT was administered to those who had malignant recurrence; 122/126 patients with MT and 53/56 with IT are alive without disease with a follow up of 8-144 months (median 56). Two patients with malignant relapse (one with sc MT, one with sc IT) died because of the progression of the disease. Another two died due to severe malformations (one MT, one IT) and three were lost to follow up (two MT, one IT). The overall survival (OS) at 10 years is 98% (95% CI 93.9-99.4); the event free survival (EFS) is 90.4% (95 CI 84.8-94.0). At Cox analysis no significant difference in EFS was found regarding age and site of the primary tumour, while females (P = 0.011), patients with grade 1-3 histology (P = 0.025) and patients with incomplete resection appeared at higher risk of death or relapse (P < 0.001), with a seven, three and eightfold increase in risk, respectively. Our data showed that incomplete resection and female gender are important risk factors for relapse or death, more so than IT histology. The number of patients treated with CT is not sufficient to evaluate the efficacy of CT in avoiding malignant relapse.
Assuntos
Neoplasias Ovarianas/epidemiologia , Teratoma/epidemiologia , Neoplasias Testiculares/epidemiologia , Distribuição por Idade , Criança , Pré-Escolar , Terapia Combinada , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Itália/epidemiologia , Masculino , Estadiamento de Neoplasias , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/terapia , Estudos Prospectivos , Teratoma/patologia , Teratoma/terapia , Neoplasias Testiculares/patologia , Neoplasias Testiculares/terapiaRESUMO
BACKGROUND: Despite the increased survival of children with solid tumors, a significant proportion of cases still relapse following treatment discontinuation, and knowledge about the long-term outcome of this selected group of patients remains incomplete. OBJECTIVE: To describe the long-term outcome of children treated for a solid tumor who relapsed after the elective end of therapy, and to explore factors associated with survival. METHODS: All patients with the selected diagnoses-Hodgkin disease (HD), neuroblastoma (NB), tumor of the central nervous system (CNS), Wilms tumor (WT), or soft tissue sarcoma (STS)-enrolled in the Italian Pediatric Off-Therapy Registry in the period 1980-1998 were evaluated. Out of 3,927 patients, 694 had relapsed after treatment suspension; 639 were available for analysis. Survival and event-free survival were estimated by the Kaplan-Meier method. The log-rank test was used to assess differences in survival among the various types of cancer considered. Multivariate Cox proportional hazards analysis was adopted to explore possible prognostic factors. RESULTS: There were 335 deaths: most of them (93%) were related to the primary cancer. The overall survival rate after relapse was 38% (95% CI 33-42) at 5 years, and 32% (95% CI 27-36%) at 15 years, while event free survival was 31% (95% CI 26-35) and 26% (95% CI 22-30%), respectively. There were significant differences according to the original diagnosis, with patients with HD doing better, and those with NB, CNS, and STS worse. No improvement of prognosis was evident over time. Post-relapse stem cell transplantation was associated with decreased risk of death only in the first year, not thereafter. CONCLUSIONS: Overall, patients with solid tumors who relapse after treatment discontinuation have a poor outcome, but significant differences exist according to the tumor types.
Assuntos
Doença de Hodgkin/diagnóstico , Neoplasias do Sistema Nervoso/diagnóstico , Neuroblastoma/diagnóstico , Sistema de Registros , Sarcoma/diagnóstico , Tumor de Wilms/diagnóstico , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Intervalo Livre de Doença , Feminino , Seguimentos , Doença de Hodgkin/terapia , Humanos , Lactente , Recém-Nascido , Itália , Masculino , Neoplasias do Sistema Nervoso/terapia , Neuroblastoma/terapia , Prognóstico , Recidiva , Fatores de Risco , Sarcoma/terapia , Transplante de Células-Tronco , Taxa de Sobrevida , Transplante Homólogo , Resultado do Tratamento , Tumor de Wilms/terapiaRESUMO
We carried out a cohort study in the Italian province of Trieste (2001 population, 242,000) to ascertain whether the risk of a subsequent primary cancer among 265 individuals diagnosed with a first malignancy at ages up to 25 years between 1971 and 1993 differed from that in the general population and to evaluate the effect of cancer family history, quantified by the family risk index (FRI), on the occurrence of second primaries. During the follow-up (median duration = 10 years; 25th-75th percentile = 2-16), 15 cohort members developed a second cancer vs. 1.60 expected for a standardized incidence ratio (SIR) of 9.4 (p < 0.0001). The overall SIR fell to 4.7 (p = 0.004) after excluding the 8 patients with well-known cancer-predisposing conditions (SIR = 300.0; p < 0.0001) and the 50 with a positive family history (FRI >/= 1.0) of malignant tumors (SIR = 20.0; p < 0.0001). Among 114 patients treated with radiotherapy and chemotherapy for their first neoplasms and not affected by predisposing disorders, 23 with a positive family history of cancer showed a 6.4-fold excess risk of second primaries (p = 0.008) compared with 91 with a negative history (FRI < 1.0). It is imperative that clinicians carefully and regularly evaluate cancer family history of young patients with malignancies. This would enable them to identify possible individual and familial features in patients at higher risk of multiple primaries and to adopt more suitable preventive and therapeutic measures.
Assuntos
Predisposição Genética para Doença , Segunda Neoplasia Primária/etiologia , Segunda Neoplasia Primária/genética , Adulto , Idade de Início , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Itália , Masculino , Anamnese , Segunda Neoplasia Primária/epidemiologia , Linhagem , Fatores de RiscoRESUMO
BACKGROUND: Childhood anaplastic large cell lymphoma (ALCL) is a well defined entity with a rather poor prognosis. Different approaches have been adopted in the treatment of ALCL in various cooperative trials, including short high-dose intensive therapy and leukemia-like protocols. In the early 1990s, the Italian Association of Pediatric Hematology and Oncology (AIEOP) initiated a multicenter trial for the treatment of ALCL based on a modified LSA2-L2 protocol. METHODS: Thirty-four consecutive eligible children with newly diagnosed ALCL were enrolled in the AIEOP LNH-92 protocol. Treatment was comprised of an induction of remission phase, followed by consolidation and maintenance for a total duration of 24 months, independently of disease stage. RESULTS: Thirty of 34 patients (88%) achieved complete disease remission and 8 patients experienced disease recurrence. With a median follow-up of 8.4 years, the probabilities of survival and event-free survival were 85% (range, 79-91%) and 65% (range, 57-73%), respectively. Therapy was well tolerated and hematologic toxicity was the most frequent toxicity. CONCLUSIONS: The leukemia-like protocol AIEOP LNH-92 was found to be an effective treatment for childhood ALCL. Its long duration may be beneficial to specific patient subgroups, but optimal treatment duration in ALCL remains to be elucidated.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/mortalidade , Adolescente , Criança , Pré-Escolar , Ciclofosfamida/uso terapêutico , Daunorrubicina/uso terapêutico , Intervalo Livre de Doença , Feminino , Humanos , Imuno-Histoquímica , Leucemia/terapia , Linfoma Difuso de Grandes Células B/patologia , Masculino , Metotrexato/uso terapêutico , Estadiamento de Neoplasias , Prednisona/uso terapêutico , Análise de Sobrevida , Resultado do Tratamento , Vincristina/uso terapêuticoRESUMO
To determine whether the occurrence of first and second primary malignancies in first-degree relatives of cancer patients aged 0-25 years (probands) differed from that in the general population, a cohort study was carried out on 860 relatives of 265 probands living in the province of Trieste, Italy. During the follow-up period (median duration = 28 years, 25th-75th percentile = 20-34), the relatives developed 103 first primary cancers vs. 88.9 expected for a standardized incidence ratio (SIR) of 1.2 (p = 0.2). Significantly elevated risks were found for melanoma in the parents of probands aged 15-25 years with melanoma (SIR = 15.0, p = 0.002), for hemolymphatic malignancies in the fathers of probands aged 0-14 years with brain tumors (SIR = 13.3, p = 0.0005) and for hemolymphatic cancers in relatives as a whole of probands aged 15-25 years with lymphomas (SIR = 4.5, p = 0.01). During the follow-up period, 7 relatives with a first primary cancer had a subsequent malignancy vs. 4.2 expected for an SIR of 1.7 (p = 0.3). Our results indicate that young cancer patients per se should not to be considered as a factor that usually increases the risk of developing malignant tumors among their first-degree relatives, except when a known cancer family syndrome or predisposition is recognized.