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1.
Tumori ; : 300891620925532, 2020 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-32462993

RESUMO

Peripheral precocious puberty (PPP) may be a paraneoplastic manifestation, associated with beta human chorionic gonadotropin (ß-hCG)-secreting tumors. We describe 2 young children with ß-hCG-secreting tumors presenting with signs of pubertal activation. In the first patient, a 16-month-old boy with hepatoblastoma, only initial signs of PPP at presentation were identifiable, with concomitant high levels of ß-hCG. Although the tumor had good response to therapy, ß-hCG levels were fluctuant until the tumor was resected surgically. The second patient, an 18-month-old boy with intracranial germ cell tumor, presented with clear signs of pubertal activation and genitalia enlargement with no initial alteration of sex hormones. In both cases, the oncologic response to therapy was good. In the first case, full remission of the pubertal signs was observed; in the second, pubertal signs were still visible 20 months after the end of treatment.

3.
Pediatr Blood Cancer ; 67(7): e28318, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32240567

RESUMO

The rapid spread of coronavirus disease 2019 epidemic in Italy, in particular in the Milan focal point, required drastic measures and led to panic in the population. While in our center we did not change our approach to the treatment of our young patients with cancer, we developed a qualitative survey to assess their perception of the risk and level of stress. The survey showed that a relatively large proportion of young patients felt personally at risk of severe complications. We believe that we need to adequately inform our patients, focusing on hygienic measures and personal protection and prompt reporting of any suspicious symptoms.

4.
Artigo em Inglês | MEDLINE | ID: mdl-32320303

RESUMO

Purpose: Adolescent and young adult patients of the Milan's Youth Project developed a new communication project aimed at young people with cancer: in a series of video tutorials called Tumorial, they talk about their day-to-day experiences, offering "survival tricks" to their peers. Methods: The project was developed during group meetings taking place every week in a dedicated room near the ward. Each meeting focused on a single topic and was led by the patients themselves, who talked about their experiences; staff members moderated the discussion and took notes, which was used as script for a video, recorded by one patient as a spokesperson. All the videos had English subtitles. Results: The project was implemented between March 2018 and June 2019 and involved 53 teenagers and young adults (15-27 years), 33 receiving treatment and 20 in follow-up when the project began. There were 23 video tutorials produced in all, with various topics, for example, school, sex, hair, privacy, social networks, fuck-ups to avoid, scars, ward companions. The videos are published on the Youth Project's YouTube channel (https://www.youtube.com/channel/UCR0EVeYMAjgJlN95tSc_iPA). Conclusion: This innovative approach to communication in the world of oncological disease in the young can be a useful tool as part of their course of care. It appears of great importance considering that social networks-and YouTube in particular-frequently provide unreliable or useless information. In making the project, patients told their innermost feelings, promoting cohesion among them. Patients and caregivers developed the project together in a significant example of cooperation.

5.
Childs Nerv Syst ; 2020 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-32222799

RESUMO

PURPOSE: There is no consensus in the literature about the impact of hydrocephalus on clinical course and overall survival of diffuse intrinsic pontine gliomas (DIPG) patients as well as about its specific treatment. Authors reviewed a series of DIPG patients to investigate factors related to the onset of hydrocephalus, its treatment, and its impact on clinical course and prognosis. METHODS: A retrospective observational study was performed enrolling pediatric patients affected by DIPG from 2008 to 2018. Clinical and radiological charts were reviewed to find patients' demographic, pathologic and radiologic features in hydrocephalic and non-hydrocephalic patients. In the hydrocephalus cohort, treatment strategy and its effectiveness and complications were analyzed. RESULTS: Ninety-four pediatric patients were enrolled in the study. Patients who developed hydrocephalus showed significantly lesser maximum axial tumor areas than patients without hydrocephalus (respectively 6.5 cm2 vs 16.45 cm2, p < 0.005). Hydrocephalus developed in 33 patients (35%) with an onset interval of 5.24 ± 1.21 months (range 3.2-7.3). The majority of hydrocephalic patients (28 cases, 90%) were treated by ventriculoperitoneal shunt, the remaining 3 patients being treated by endoscopic third ventriculostomy. Mean overall survival was 16.6 months ± 20 months without significative difference between the groups. CONCLUSION: The onset of hydrocephalus occurs in the first moths of the disease story and found a negative correlation with tumor maximal axial diameter. Early treatment of hydrocephalus presents a very low complications rate with satisfying clinical outcome, as it allows the patients to continue the neurooncological therapies being a part of the treatment armamentarium instead of a palliative solution.

6.
Eur J Pediatr ; 2020 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-32140854

RESUMO

The incidence of cancer in children with intellectual disability has been poorly documented. We report our experience of treating children and adolescents with cancer and intellectual disability (40 patients), from 2004 to 2018. A treatment-sparing approach was adopted for 6 patients with severe intellectual impairment to minimize toxicity: a child with postpartum asphyxia and medulloblastoma did not receive radiotherapy; 1 patient with mitochondrial encephalopathy and a testicular germ cell tumor did not receive bleomycin and lung metastasectomy; 2 patients (1 with Down + West syndrome + Wilms tumor (WT) and 1 with Denys-Drash syndrome + WT) did not receive vincristine; 1 child with corpus callosum agenesis and anaplastic ependymoma did not receive chemotherapy; 1 child with structural chromosomal aberrations and a primitive neuro-ectodermal tumor received personalized chemotherapy. Heminephrectomy was performed in 4 patients with WT to preserve their kidney function. We found no statistically significant correlation between relapse or mortality rates and the use of a treatment-sparing approach. The 5-year overall survival (OS) and event-free survival (EFS) rates were 84.5% and 66.1% as opposed to 82.5% and 46.9%, respectively, for patients in our usual-treatment and treatment-sparing groups.Conclusion: We only opted for a treatment-sparing approach for patients with severe disabilities, and their OS was in line with that of children without intellectual disability.What is Known:• There are few reports on children/adolescents with cancer and intellectual disability (ID).• It is not clear how to manage them and whether a treatment sparing should be considered, especially in the case of severe disability.What is New:• Most patients received the standard cancer treatment and only in the case of severe disability, a therapeutic saving approach was applied.• No statistically significant correlations between relapse/mortality rates and the use of a treatment-sparing approach were found.

7.
Childs Nerv Syst ; 36(4): 697-704, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31848724

RESUMO

PURPOSE: The diagnosis of diffuse intrinsic pontine glioma (DIPG) is based largely on a combination of clinical and radiological findings due to the difficulty of obtaining a biopsy. An accurate evaluation of magnetic resonance imaging (MRI) scans is consequently essential. Recent analyses on the genomic landscape of DIPG revealed recurrent mutations in the H3F3A and HIST1H3B histone genes. We reviewed cases with available tumor tissue from institutional DIPG series to ascertain the consistency between their histo-molecular findings and clinical-radiological features. METHODS: We conducted a radiological and pathological central review of 22 cases enrolled in institutional DIPG trials. We performed immunohistochemical analyses to detect H3F3A/HIST1H3B K27M mutations, histone trimethylation, and EZH2 expression. Mutational analysis was performed for ACVR1, H3F3A, and HIST1H3B genes. RESULTS: Patients' median age at diagnosis was 8 years, and their median overall survival was 11 months. Nineteen/22 cases (86%) showed evidence of K27M mutation on immunohistochemistry and/or mutation analysis. Histone trimethylation expression was low or lacking in these mutated cases. Sequence analysis revealed 13 cases with H3F3A and 1 case with HIST1H3B K27M mutation. There was no significant difference in EZH2 expression between the K27M mutant and wild-type DIPGs. Upon external, blinded MRI re-evaluation one lesion not consistent with DIPG showed no evidence of K27M mutation and retained histone trimethylation expression. CONCLUSION: In conclusion, our study demonstrates a high frequency of histone K27M mutations in DIPG when MRI features are carefully assessed, thus confirming the consistency of imaging with biological markers in our institutional series of DIPG.

8.
Cancer Metastasis Rev ; 38(4): 683-694, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31797181

RESUMO

Wilms tumor (or nephroblastoma), rhabdomyosarcoma, and medulloblastoma, common embryonal tumors in children, can occasionally occur in adults, for whom survival is significantly inferior than pediatric patients. Available data on adults with Wilms tumor consist of case or case series reports. Among other factors, the unfamiliarity of adult oncologists and pathologists with nephroblastoma and consequent delays in initiating the appropriate risk-adapted chemotherapy may negatively influence outcomes. The survival decrement in adults with rhabdomyosarcoma has been attributed to the lack of centralized care, the inconsistent use of standard protocol-driven multimodal therapy, and lower chemotherapy tolerance in adult patients. In children with medulloblastoma, evidence from randomized clinical trials has led to risk-tailored therapies tuned on histology, extent of initial disease, and biological features. Such refinements are still missing for adults due to the lack of similar trials and studies that might provide the same or a different understanding regarding patients' individual prognosis, treatment morbidity, and quality of life. Recent experiences have suggested that applying or adjusting pediatric protocols to adult patients with these tumors is feasible and can improve survival. Here, we provide an evaluation of the current evidence for the management of Wilms tumor, rhabdomyosarcoma, and medulloblastoma arising in adults. This review aims to promote the referral of adolescents and adults with pediatric tumors to pediatric centers for inclusion into pediatric protocols, or into protocols and studies specifically designed for that age group with the cooperation between pediatric and adult oncologists.


Assuntos
Neoplasias Cerebelares/diagnóstico , Neoplasias Renais/diagnóstico , Meduloblastoma/diagnóstico , Rabdomiossarcoma/diagnóstico , Tumor de Wilms/diagnóstico , Adulto , Fatores Etários , Neoplasias Cerebelares/tratamento farmacológico , Humanos , Neoplasias Renais/tratamento farmacológico , Meduloblastoma/tratamento farmacológico , Rabdomiossarcoma/tratamento farmacológico , Tumor de Wilms/tratamento farmacológico
9.
Tumori ; 105(6): NP75-NP78, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31600120

RESUMO

This study reports a case series of patients with upper limb neuromotor deficits following pediatric central nervous system tumor and treated with rehabilitative therapy according to action observation therapy (AOT). AOT is based on the "mirror neurons" system and had positive results in various non-oncologic neurologic pathologies. This study is the first experience in the oncology field, and included 6 patients, 4 of whom were fully evaluated at 6-month follow-up. In all patients, therapy showed improvement in all assessment tests. These promising results lead to further studies to confirm their effectiveness.


Assuntos
Neoplasias do Sistema Nervoso Central/complicações , Reabilitação Neurológica , Doenças Neuromusculares/etiologia , Doenças Neuromusculares/reabilitação , Extremidade Superior/fisiopatologia , Adolescente , Adulto , Feminino , Humanos , Masculino , Reabilitação Neurológica/métodos , Doenças Neuromusculares/diagnóstico , Doenças Neuromusculares/terapia , Resultado do Tratamento , Adulto Jovem
10.
Eur J Nucl Med Mol Imaging ; 46(8): 1685-1694, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31030232

RESUMO

PURPOSE: The aim of this study was to investigate MRI-derived diffusion weighted imaging (DWI), 1H-MR spectroscopy (1H-MRS) and arterial spin labeling (ASL) perfusion imaging in comparison with 18F-dihydroxyphenylalanine (DOPA) PET with respect to diagnostic evaluation of pediatric diffuse midline gliomas (DMG) H3K27M-mutant and wild-type. METHODS: We retrospectively analyzed 22 pediatric patients with DMG histologically proved and molecularly classified as H3K27M-mutant (12 subjects) and wild-type (10 subjects) who underwent DWI, 1H-MRS, and ASL performed within 2 weeks of 18F-DOPA PET. DWI-derived relative minimum apparent diffusion coefficient (rADC min), 1H-MRS data [choline/N-acetylaspartate (Cho/NAA), choline/creatine (Cho/Cr), and presence of lactate] and relative ASL-derived cerebral blood flow max (rCBF max) were compared with 18F-DOPA uptake Tumor/Normal tissue (T/N) and Tumor/Striatum (T/S) ratios, and correlated with histological and molecular features of DMG. Statistics included Pearson's chi-square and Mann-Whitney U tests, Spearman's rank correlation and receiver operating characteristic (ROC) analysis. RESULTS: The highest degrees of correlation among different techniques were found between T/S, rADC min and Cho/NAA ratio (p < 0.01), and between rCBF max and rADC min (p < 0.01). Significant differences between histologically classified low- and high-grade DMG, independently of H3K27M-mutation, were found among all imaging techniques (p ≤ 0.02). Significant differences in terms of rCBF max, rADC min, Cho/NAA and 18F-DOPA uptake were also found between molecularly classified mutant and wild-type DMG (p ≤ 0.02), even though wild-type DMG included low-grade astrocytomas, not present among mutant DMG. When comparing only histologically defined high-grade mutant and wild-type DMG, only the 18F-DOPA PET data T/S demonstrated statistically significant differences independently of histology (p < 0.003). ROC analysis demonstrated that T/S ratio was the best parameter for differentiating mutant from wild-type DMG (AUC 0.94, p < 0.001). CONCLUSIONS: Advanced MRI and 18F-DOPA PET characteristics of DMG depend on histological features; however, 18F-DOPA PET-T/S was the only parameter able to discriminate H3K27M-mutant from wild-type DMG independently of histology.

11.
J Neurooncol ; 140(2): 457-465, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30109673

RESUMO

PURPOSE: The aims of patients' radiological surveillance are to: ascertain relapse; apply second-line therapy; accrue patients in phase 1/2 protocols if second-line therapy is not standardized/curative; and assess/treat iatrogenic effects. To lessen the emotional and socioeconomic burdens for patients and families, we ideally need to establish whether scheduled radiological surveillance gives patients a better outcome than waiting for symptoms and signs to appear. METHODS: We analyzed a prospective series of 160 newly-diagnosed and treated pediatric/adolescent patients with intracranial ependymoma, comparing patients with recurrent disease identified on scheduled MRI (the RECPT group; 34 cases) with those showing signs/symptoms of recurrent disease (the SYMPPT group; 16 cases). The median follow-up was 67 months. RESULTS: No significant differences emerged between the two groups in terms of gender, age, tumor grade/site, shunting, residual disease, or type of relapse (local, distant, or concomitant). The time to relapse (median 19 months; range 5-104) and the MRI follow-up intervals did not differ between the SYMPPT and RECPT groups. The presence of signs/symptoms was an unfavorable factor for overall survival (OS) after recurrence (5-year OS: 8% vs. 37%, p = 0.001). On multivariable analysis, an adjusted model confirmed a significantly worse OS in the SYMPPT than in the RECPT patients. CONCLUSIONS: Symptomatic relapses carried a significantly worse survival for ependymoma patients than recurrences detected by MRI alone. It would therefore be desirable to identify recurrences before symptoms develop. Radiological follow-up should be retained in ependymoma patient surveillance because there is a chance of salvage treatment for relapses found on MRI.


Assuntos
Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/terapia , Ependimoma/diagnóstico , Ependimoma/terapia , Adolescente , Neoplasias Encefálicas/mortalidade , Criança , Pré-Escolar , Protocolos Clínicos , Ependimoma/mortalidade , Feminino , Seguimentos , Humanos , Imagem por Ressonância Magnética , Masculino , Recidiva Local de Neoplasia , Prognóstico , Estudos Prospectivos
12.
Childs Nerv Syst ; 34(7): 1291-1298, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29725826

RESUMO

BACKGROUND: Primary pediatric extraspinal sacrococcygeal ependymoma (ESE) is a very rare disease, poorly described in literature, whose diagnostic, therapeutic, and follow-up approach is still controversial. METHODS: We describe six cases of pediatric ESE treated at Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers in Italy since 1983, with a review of the literature. RESULTS: All six patients had primary sacrococcygeal disease (two presacral and four subcutaneous) with median age of 10 years. Three patients were males, and two of them are metastatic at diagnosis; 3/6 had myxopapillary ependymoma grade I and 3/6 had classic ependymoma grade II. Five patients underwent surgical resection with complete removal only in one case with coccygectomy. Adjuvant chemoradiotherapy was administered to one metastatic patient obtaining a complete remission. Two patients relapsed at 3 and 8 years from diagnosis: they were treated with salvage chemotherapy (high-dose sequential chemotherapy with myeloablative regimen in one case), surgery, and radiotherapy achieving complete remission (CR). All six patients are in complete continuous remission (CCR) at a median follow-up of 12.8 years. CONCLUSIONS: Pediatric patients with this peculiar disease need to be referred to specialized pediatric cancer centers that can provide multidisciplinary treatment after a centralized pathology review. Our experience highlights the role of chemotherapy and radiotherapy in adjuvant and relapse setting. The final prognosis is relatively optimistic, but with a careful follow-up due to the high risk of recurrence.


Assuntos
Ependimoma/patologia , Neoplasias de Tecidos Moles/patologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Itália , Masculino , Região Sacrococcígea , Tela Subcutânea
13.
J Neurooncol ; 138(3): 679-680, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29767306

RESUMO

The therapeutic experience reported in the paper was conceived after the use of nimotuzumab and radiotherapy (BSCPED-05 international multicentric trial, EUDRACT 2005-003100-11) in 2009 when we decided to explore the activity of the same combination plus vinorelbine (see the paper for the rationale).

14.
Med Oncol ; 34(12): 191, 2017 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-29094224

RESUMO

To improve the poor prognosis for children with metastatic osteosarcoma (OS), interleukin-2 (IL-2) was added to the standard treatment due to its capacity to activate lymphocytes and differentiate lymphocyte subsets into lymphokine-activated killer (LAK) cells that are capable of recognizing and killing various tumor cells. This study concerns a cohort of unselected patients aged < 18 years with metastatic OS, who were treated with IL-2, high-dose methotrexate, doxorubicin, cisplatin, ifosfamide, LAK reinfusion, and surgery, between 1995 and 2010. Thirty-five patients aged 4-17 years were involved. Thirty-two of the 35 patients underwent surgery on their primary tumor, and 25 had surgery on lung metastases too. Twenty-seven patients received IL-2 plus LAK reinfusion. The median follow-up was 130 months (77-228), and the 3-year event-free and overall survival rates were 34.3 and 45.0%, respectively. Eleven patients remained alive, all of whom achieved a complete surgical removal of the primary tumor and lung metastases (1 patient did not receive lung resections due to complete lung metastases remission). Patients who had a complete surgical remission of the primary and metastatic sites and who responded well to chemotherapy had a better event-free survival. These results confirm the importance of complete surgical remission and point to a noteworthy (though still be ameliorate) survival rate in our series of patients, underling a potential role for immunotherapy with IL-2 and LAK/NK cell activation.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Interleucina-2/uso terapêutico , Osteossarcoma/tratamento farmacológico , Adolescente , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Neoplasias Ósseas/terapia , Criança , Pré-Escolar , Cisplatino/administração & dosagem , Doxorrubicina/administração & dosagem , Feminino , Humanos , Ifosfamida/administração & dosagem , Imunoterapia/métodos , Interleucina-2/administração & dosagem , Neoplasias Pulmonares/tratamento farmacológico , Neoplasias Pulmonares/secundário , Masculino , Osteossarcoma/mortalidade , Osteossarcoma/patologia , Osteossarcoma/terapia , Estudos Prospectivos , Taxa de Sobrevida
15.
Tumori ; 103(6): 566-571, 2017 Nov 23.
Artigo em Inglês | MEDLINE | ID: mdl-28708228

RESUMO

PURPOSE: Cardiac late effects are responsible for a significant burden of mortality and morbidity among pediatric Hodgkin's lymphoma (HL) survivors (HLS). The aim of our study was to assess clinical and subclinical cardiac sequelae in a cohort of childhood HLS treated in the 1980s with doxorubicin, bleomycin, vinblastine, and dacarbazine (the ABVD regimen) and limited-field radiotherapy (RT). METHODS: We retrospectively examined a series of HLS treated from 1979 to 1989. We searched for subtle cardiac abnormalities in a subgroup of asymptomatic individuals, who underwent rest and exercise echocardiography at least 20 years after completing their therapies. Their cardiac assessment included physical examination, electrocardiogram (ECG), and resting and postexercise echocardiograms. RESULTS: On thorough cardiac assessment a mean of 21 years after their diagnosis, none of the 53 unselected asymptomatic HLS showed physical signs or significant ECG abnormalities during or after the stress echo test. Twenty-two (41%) of the 53 patients revealed valvular abnormalities, with mitral regurgitation in 28%, aortic regurgitation in 9%, and both in 4%. No significant myocardial dysfunction as a result of previous combined doxorubicin treatment and chest RT was identified. Only 2 individuals had mild pericardial alterations. CONCLUSIONS: The present study shows that long-term cardiac effects are common in HLS treated with the ABVD regimen and RT. The most frequent complications observed in this sample were essentially coronary artery disease and valvular abnormalities. None of the survivors in this sample showed overt congestive heart failure, a finding in contrast with larger studies.


Assuntos
Quimiorradioterapia/efeitos adversos , Cardiopatias/epidemiologia , Cardiopatias/etiologia , Doença de Hodgkin/terapia , Sobreviventes/estatística & dados numéricos , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Bleomicina/efeitos adversos , Criança , Pré-Escolar , Dacarbazina/efeitos adversos , Doxorrubicina/efeitos adversos , Feminino , Coração/efeitos dos fármacos , Coração/efeitos da radiação , Humanos , Masculino , Estudos Retrospectivos , Vimblastina/efeitos adversos
16.
J Neurooncol ; 131(2): 349-357, 2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27770281

RESUMO

To assess the long-term safety of administering growth hormone (GH) in patients with GH deficiency due to treatment for childhood medulloblastoma and primitive neuroectodermal tumor (PNET). Data were retrospectively retrieved on children receiving GH supplementation, assessing their disease-free and overall survival outcomes and risk of secondary malignancies using Kaplan-Meier and Cox models. Overall 65 children were consecutively collected from May 1981 to April 2013. All patients had undergone craniospinal irradiation (total dose 18-39 Gy), and subsequently received GH for a median (interquartile range, IQR) of 81 (50.6-114.9) months. At a median (IQR) of 122.4 months (74.4-149.5) after the end of their adjuvant cancer treatment, two patients (3 %) experienced recurrent disease and 8 (12.3 %) developed secondary malignancies, all but one of them (an osteosarcoma) related to radiation exposure and occurring within the radiation fields. There was no apparent correlation between the administration of GH replacement therapy (or its duration) and primary tumor relapse or the onset of secondary malignancies [HR: 1.01 (95 % CI: 0.98, 1.03) for every additional 12 months of GH supplementation; p = 0.36). At univariate analysis, the large cell or anaplastic medulloblastoma subtype, metastases and myeloablative chemotherapy correlated with a higher risk of secondary malignancies (p < 0.1), but multivariate analysis failed to identify any factors independently associated with this risk. Our data supports once more the safety of long-term GH replacement therapy in children treated for medulloblastoma/PNET, previously reported in larger data sets. The neurooncology community now need to warrant large-scale meta-analyses or international prospective trials in order to consolidate our knowledge of factors other than GH, such as genetic predisposition, high-grade/metastatic disease, high-dose chemotherapy and era of treatment, in promoting the occurrence of secondary malignancies.


Assuntos
Neoplasias Encefálicas/tratamento farmacológico , Hormônio do Crescimento/efeitos adversos , Terapia de Reposição Hormonal/efeitos adversos , Meduloblastoma/tratamento farmacológico , Tumores Neuroectodérmicos Primitivos/tratamento farmacológico , Criança , Feminino , Hormônio do Crescimento/uso terapêutico , Humanos , Estimativa de Kaplan-Meier , Masculino , Estudos Retrospectivos , Resultado do Tratamento
17.
Pediatr Blood Cancer ; 63(12): 2197-2204, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27554940

RESUMO

BACKGROUND: Various projects dedicated specifically to adolescents and young adults (AYA) with cancer have been developed in recent years. A critical aspect of such programs is the ability to demonstrate its value, and therefore how to measure desired outcomes. METHODS: A list of metrics to consider for demonstrating the advantages of an AYA program was identified and used to assess the activity of the Youth Project operating at the Pediatric Oncology Unit of the Istituto Nazionale Tumori in Milan. RESULTS: The number of newly diagnosed AYA patients seen at the Unit has increased since the formal launch of the Youth Project, from 65 to 81.2 cases/year. Concerning the 78 AYA patients presenting with malignant neoplasms in 2015, 82% were included in clinical trials (the other 18% in prospective observational studies). Fertility preservation measures were implemented for 59% of AYA patients considered at risk, and specific psychological support was provided in 70.6% of cases; 72.5% of patients actively participated in support activities. Other parameters considered were a preliminary satisfaction questionnaire administered to patients and the program's scientific recognition and acknowledgment by the community. CONCLUSIONS: The study proposed a number of potentially reproducible, practical parameters to consider in assessing the value of a program dedicated to AYA. These metrics were examined in terms of the activities of our Youth Project, and confirmed its efficacy. To be sustainable over time, AYA projects have to be accepted as a standard of care at the community and government levels.


Assuntos
Neoplasias/terapia , Adolescente , Adulto , Feminino , Humanos , Masculino , Apoio Social , Padrão de Cuidado , Adulto Jovem
18.
Cancer Med ; 5(9): 2359-67, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27485192

RESUMO

The purpose of the study was to determine if abdomen/pelvis computed tomography (CT) can be safety omitted in the initial staging of a subgroup of children affected by Hodgkin Lymphoma (HL). Every participating center of A.I.E.O.P (Associazione Italiana di Ematologia ed Oncologia Pediatrica) sent local staging reports of 18F-fluorodeoxyglucose positron emission tomography (PET) and abdominal ultrasound (US) along with digital images of staging abdomen/pelvis CT to the investigation center where the CT scans were evaluated by an experienced pediatric radiologist. The local radiologist who performed the US was unaware of local CT and PET reports (both carried out after US), and the reviewer radiologist examining the CT images was unaware of local US, PET and CT reports. A new abdominal staging of 123 patients performed on the basis of local US report, local PET report, and centralized CT report was then compared to a simpler staging based on local US and PET. No additional lesion was discovered by CT in patients with abdomen/pelvis negativity in both US and PET or isolated spleen positivity in US (or US and PET), and so it seems that in the initial staging, abdomen/pelvis CT can be safety omitted in about 1/2 to 2/3 of children diagnosed with HL.


Assuntos
Abdome/patologia , Doença de Hodgkin/diagnóstico por imagem , Doença de Hodgkin/patologia , Pelve/patologia , Tomografia Computadorizada por Raios X , Feminino , Humanos , Linfonodos/patologia , Masculino , Imagem Multimodal , Estadiamento de Neoplasias , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Tomografia por Emissão de Pósitrons , Sensibilidade e Especificidade , Tomografia Computadorizada por Raios X/métodos
19.
Neuro Oncol ; 18(10): 1451-60, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-27194148

RESUMO

BACKGROUND: This prospective study stratified patients by surgical resection (complete = NED vs incomplete = ED) and centrally reviewed histology (World Health Organization [WHO] grade II vs III). METHODS: WHO grade II/NED patients received focal radiotherapy (RT) up to 59.4 Gy with 1.8 Gy/day. Grade III/NED received 4 courses of VEC (vincristine, etoposide, cyclophosphamide) after RT. ED patients received 1-4 VEC courses, second-look surgery, and 59.4 Gy followed by an 8-Gy boost in 2 fractions on still measurable residue. NED children aged 1-3 years with grade II tumors could receive 6 VEC courses alone. RESULTS: From January 2002 to December 2014, one hundred sixty consecutive children entered the protocol (median age, 4.9 y; males, 100). Follow-up was a median of 67 months. An infratentorial origin was identified in 110 cases. After surgery, 110 patients were NED, and 84 had grade III disease. Multiple resections were performed in 46/160 children (28.8%). A boost was given to 24/40 ED patients achieving progression-free survival (PFS) and overall survival (OS) rates of 58.1% and 68.7%, respectively, in this poor prognosis subgroup. For the whole series, 5-year PFS and OS rates were 65.4% and 81.1%, with no toxic deaths. On multivariable analysis, NED status and grade II were favorable for OS, and for PFS grade II remained favorable. CONCLUSIONS: In a multicenter collaboration, this trial accrued the highest number of patients published so far, and results are comparable to the best single-institution series. The RT boost, when feasible, seemed effective in improving prognosis. Even after multiple procedures, complete resection confirmed its prognostic strength, along with tumor grade. Biological parameters emerging in this series will be the object of future correlatives and reports.


Assuntos
Neoplasias Encefálicas/terapia , Quimiorradioterapia Adjuvante/métodos , Ependimoma/terapia , Procedimentos Neurocirúrgicos/métodos , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Encefálicas/mortalidade , Neoplasias Encefálicas/patologia , Quimiorradioterapia Adjuvante/mortalidade , Criança , Pré-Escolar , Ciclofosfamida/administração & dosagem , Intervalo Livre de Doença , Ependimoma/mortalidade , Ependimoma/patologia , Etoposídeo/administração & dosagem , Feminino , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Procedimentos Neurocirúrgicos/mortalidade , Radioterapia , Resultado do Tratamento , Vincristina/administração & dosagem
20.
Pediatr Blood Cancer ; 63(3): 479-85, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26797893

RESUMO

BACKGROUND: The potential impact of diagnostic delays on patients' outcomes is a debated issue in pediatric oncology and discordant results have been published so far. We attempted to tackle this issue by analyzing a prospective series of 351 consecutive children and adolescents with solid malignancies using innovative statistical tools. METHODS: To address the nonlinear complexity of the association between symptom interval and overall survival (OS), a regression tree algorithm was constructed with sequential binary splitting rules and used to identify homogeneous patient groups vis-à-vis functional relationship between diagnostic delay and OS. RESULTS: Three different groups were identified: group A, with localized disease and good prognosis (5-year OS 85.4%); group B, with locally or regionally advanced, or metastatic disease and intermediate prognosis (5-year OS 72.9%), including neuroblastoma, Wilms tumor, non-rhabdomyosarcoma soft tissue sarcoma, and germ cell tumor; and group C, with locally or regionally advanced, or metastatic disease and poor prognosis (5-year OS 45%), including brain tumors, rhabdomyosarcoma, and bone sarcoma. The functional relationship between symptom interval and mortality risk differed between the three subgroups, there being no association in group A (hazard ratio [HR]: 0.96), a positive linear association in group B (HR: 1.48), and a negative linear association in group C (HR: 0.61). CONCLUSIONS: Our analysis suggests that at least a subset of patients can benefit from an earlier diagnosis in terms of survival. For others, intrinsic aggressiveness may mask the potential effect of diagnostic delays. Based on these findings, early diagnosis should remain a goal for pediatric cancer patients.


Assuntos
Neoplasias/diagnóstico , Neoplasias/terapia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Neoplasias/mortalidade , Estudos Prospectivos , Fatores de Tempo , Resultado do Tratamento
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