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1.
Med Biol Eng Comput ; 2020 Feb 11.
Artigo em Inglês | MEDLINE | ID: mdl-32048135

RESUMO

Survival of pediatric patients with brain tumor has increased over the past 20 years, and increasing evidence of iatrogenic toxicities has been reported. In follow-ups, images are acquired at different time points where substantial changes of brain morphology occur, due to childhood physiological development and treatment effects. To address the image registration complexity, we propose two multi-metric approaches (Mplus, Mdot), combining mutual information (MI) and normalized gradient field filter (NGF). The registration performance of the proposed metrics was assessed on a simulated dataset (Brainweb) and compared with those obtained by MI and NGF separately, using mean magnitude and mean angular errors. The most promising metric (Mplus) was then selected and tested on a retrospective dataset comprising 45 pediatric patients who underwent focal radiotherapy for brain cancer. The quality of the realignment was scored by a radiation oncologist using a perceived misalignment metric (PM). All patients but one were assessed as PM ≤ 2 (good alignment), but the remaining one, severely affected by hydrocephalus and pneumocephalus at the first MRI acquisition, scored PM = 5 (unacceptable). These preliminary findings suggest that Mplus might improve the registration accuracy in complex applications such as pediatric oncology, when data are acquired throughout the years of follow-up, and is worth investigating. Graphical abstract Graphical abstract showing the clinical workflow of the overall registration procedure including the three rigid steps, the fourth deformable step, the reference MRI and the registered MRI as well as the contoured ROIs. The registration performance is assessed by means of the Perceived Misalignment score (PM).

2.
Pediatr Hematol Oncol ; : 1-12, 2020 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-32022619

RESUMO

Objective. The aim of the present study is to understand which areas of sexuality were regarded as most important and/or problematic among adolescents with cancer.Methods. A questionnaire was administered to adolescent and young adult patients who had been receiving treatments at the Pediatric Oncology Unit of our Institution, for at least 2 months, and those in follow-up who had completed their treatments no more than two years previously. The questionnaire was devised to investigate patients' experiences in various areas, i.e. personal relations, sexual relations; functional aspects, body image, and communication.Results. Questionnaires were given to 70 patients and completed by 66. As main results, the survey showed that disease and treatment might negatively affect patients' way of relating with others (53% of cases) and their body image (56%), though their sexual desire remains unchanged (69.7%). Most patients (67%) reported not having the chance to talk to someone about having sex while receiving their treatments; 79% of patients felt this topic deserved more attention.Conclusion. This study promotes the discussion of an important topic for young people with cancer, which is inadequately addressed today. When establishing an age-specific model of care, aspects related to sexuality should be considered and managed.

3.
J Neurosurg Pediatr ; : 1-9, 2020 Jan 17.
Artigo em Inglês | MEDLINE | ID: mdl-31952041

RESUMO

OBJECTIVE: Extensive resection of a tumor in the posterior fossa in children is associated with the risk of neurological deficits. The objective of this study was to prospectively evaluate the short-term neurological morbidity in children after medulloblastoma surgery and relate this to the tumor's growth pattern and to the extent of resection. METHODS: In 160 patients taking part in the HIT-SIOP PNET 4 (Hyperfractionated Versus Conventionally Fractionated Radiotherapy in Standard Risk Medulloblastoma) trial, neurosurgeons prospectively responded to questions concerning the growth pattern of the tumor they had resected. The extent of resection (gross, near, or subtotal) was evaluated using MRI. The patients' neurological status before resection and around 30 days after resection was recorded. RESULTS: Invasive tumor growth, defined as local invasion in the brain or meninges, cranial nerve, or major vessel, was reported in 58% of the patients. After surgery almost 70% of all patients were affected by one or several neurological impairments (e.g., impaired vision, impaired extraocular movements, and ataxia). However, this figure was very similar to the preoperative findings. Invasive tumor growth implied a significantly higher number of impairments after surgery (p = 0.03) and greater deterioration regarding extraocular movements (p = 0.012), facial weakness (p = 0.048), and ataxia in the arms (p = 0.014) and trunk (p = 0.025) compared with noninvasive tumor growth. This deterioration was not dependent on the extent of resection performed. Progression-free survival (PFS) at 5 years was 80% ± 4% and 76% ± 5% for patients with invasive and noninvasive tumor growth, respectively, with no difference in the 5-year PFS for extent of resection. CONCLUSIONS: Preoperative neurological impairments and invasive tumor growth were strong predictors of deterioration in short-term neurological outcome after medulloblastoma neurosurgery, whereas the extent of resection was not. Neither tumor invasiveness nor extent of resection influenced PFS. These findings support the continuation of maximal safe resection in medulloblastoma surgery where functional risks are not taken in areas with tumor invasion.

4.
Clin Cancer Res ; 2020 Jan 10.
Artigo em Inglês | MEDLINE | ID: mdl-31924736

RESUMO

BACKGROUND: The HERBY trial evaluated the benefit of the addition of the antiangiogenic agent Bevacizumab (BEV) to radiotherapy/Temozolomide (RT/TMZ) in pediatric patients with newly diagnosed non-brainstem high-grade glioma (HGG). The work presented here aims to correlate imaging characteristics and outcome measures with pathological and molecular data. METHODS: Radiological, pathological and molecular data were correlated with trial clinical information to retrospectively re-evaluate event free and overall survival. RESULTS: One-hundred thirteen patients were randomized to the RT/TMZ arm (n =54) or the RT/TMZ+BEV (BEV arm; n =59). The tumor arose in the cerebral hemispheres in 68 patients (Cerebral group) and a midline location in 45 cases (Midline group). Pathological diagnosis was available in all cases and molecular data in 86/113. H3 K27M histone mutations were present in 23/32 Midline cases and H3 G34R/V mutations in 7/54 Cerebral cases. Total/near-total resection occurred in 44/68 (65%) Cerebral cases but only 5/45 (11%) Midline cases (p <0.05). Leptomeningeal metastases (27 cases, 13 with subependymal spread) at relapse were more frequent in Midline (17/45) than Cerebral tumors (10/68, p <0.05). Mean OS (14.1 months) and EFS (9.0 months) in Midline tumors were significantly lower than mean OS (20.7 months) and EFS (14.9 months) in Cerebral tumors (p <0.05). Pseudoprogression occurred in 8/111 (6.2%) cases. CONCLUSIONS: This study has shown that the poor outcome of midline tumors (compared to cerebral) may be related to 1) lesser surgical resection, 2) H3 K27M histone mutations, and 3) higher leptomeningeal dissemination.

5.
Pediatr Blood Cancer ; 67(3): e28110, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31802619

RESUMO

BACKGROUND: APC gene pathogenic variants are characterized by a lifetime risk of nearly 100% to develop a colorectal carcinoma. International guidelines suggest a prophylactic surgery in the second decade. METHODS: A descriptive analysis was performed evaluating a surgical series of adolescent patients with familial adenomatous polyposis (FAP) enrolled in the prospectively maintained hereditary polyposis registry. RESULTS: Thirty-eight adolescent patients (median age 16 years; range, 7-19) underwent laparoscopic prophylactic surgery. APC gene pathogenic variants were detected in all patients, and six patients were proband. No patients were converted to open surgery. Median postoperative stay was five days (4-16). Early postoperative complications were one dural puncture and one anastomotic leakage. Regarding late complications, we observed one patient having small bowel obstruction 56 months after surgery. Pathological reports showed one patient with pTis adenocarcinoma in two separate sites; 33 patients with low-grade dysplasia, four with high-grade dysplasia. One patient developed a desmoid tumor 37 months after surgery. After a median follow-up of 40.5 months, no patients died or had a second abdominal surgery because of cancer in rectal stump. CONCLUSIONS: Rectal sparing surgery was the first choice in the major respect of patients' quality of life. Laparoscopic prophylactic surgery for FAP is well accepted from adolescents. It represents a safe option due to the low incidence of post-surgical desmoids and quick postoperative recovery.

6.
Cancer Metastasis Rev ; 38(4): 683-694, 2019 Dec.
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.

7.
Childs Nerv Syst ; 2019 Dec 12.
Artigo em Inglês | MEDLINE | ID: mdl-31832765

RESUMO

PURPOSE: Effects of tumor location on cognitive performance of patients with brain tumor are controversial: some studies reported higher risks related to supratentorial locations, some to infratentorial locations, and still others did not find any differences. We aimed to address this issue by comparing school-aged children with supratentorial or infratentorial tumor with respect not only to cognitive outcomes but also to the associations between core cognitive domains and academic abilities. METHODS: 32 children with infratentorial tumor and 22 with supratentorial tumor participated in the study. To detect relationships among cognitive domains, we tested which neuropsychological variable(s) predicted academic skills, controlling for the effects of radiotherapy and time since diagnosis. RESULTS: Radiotherapy and time since diagnosis, but not tumor location, predicted cognitive outcomes. Radiotherapy negatively influenced attention and executive functioning, as well as reading speed and arithmetic operations accuracy. Unexpectedly, longer time since diagnosis was associated with improvement in attention and reading speed. Tumor location showed an effect on the relationships between core cognitive domains and academic skills: verbal and visual-spatial memory influenced reading and mathematical performance in supratentorial patients; in infratentorial patients, an only effect of visual-spatial memory on mathematical performance was detected. CONCLUSIONS: Tumor location seems not to influence cognitive performance, while radiotherapy constitutes a key risk factor for cognitive impairment. Attentional and reading abilities may improve over time, possibly due to the weakening of cancer care effects. Different patterns of cognitive associations seem to characterize supratentorial and infratentorial patients, probably associated with different neuroplastic reorganization processes after tumor occurrence.

8.
Childs Nerv Syst ; 2019 Dec 17.
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.

9.
Int J Mol Sci ; 20(22)2019 Nov 19.
Artigo em Inglês | MEDLINE | ID: mdl-31752446

RESUMO

Rhabdomyosarcoma (RMS), the most common soft tissue sarcoma of childhood and adolescence, is a rare but aggressive malignancy that originates from immature mesenchymal cells committed to skeletal muscle differentiation. Although RMS is, generally, responsive to the modern multimodal therapeutic approaches, the prognosis of RMS depends on multiple variables and for some patients the outcome remains dismal. Further comprehension of the molecular and cellular biology of RMS would lead to identification of novel therapeutic targets. MicroRNAs (miRNAs) are small non-coding RNAs proved to function as key regulators of skeletal muscle cell fate determination and to play important roles in RMS pathogenesis. The purpose of this review is to better delineate the role of miRNAs as a biomarkers or functional leaders in RMS development, so to possibly elucidate some of RMS molecular mechanisms and potentially therapeutically target them to improve clinical management of pediatric RMS.

10.
Artigo em Inglês | MEDLINE | ID: mdl-31660779

RESUMO

Purpose: Adolescents (15-19 years) with soft tissue sarcomas (STS) have worse survival than children. One reason is the former's limited access to expert centers. We investigated where adolescents with STS are treated in Italy, analyzing hospital discharge records (HDRs) countrywide. Methods: We applied to the Health Ministry to obtain the HDRs of all adolescents hospitalized in 2002-2015. We excluded prevalent cases hospitalized with STS in 2002-2006 to obtain a cohort of incident cases 2007-2014. We defined main treatments observing 12 months from diagnosis. Thus, the cohorts end in 2014 rather than 2015. We computed "hospital volume" as the number of adolescents treated by a hospital in 8 years. Patient migration across geographical areas was investigated comparing patients' place of residence and of hospitalization. Results: We identified 381 adolescents with STS, 63% of them were treated at AIEOP (Associazione Italiana Ematologia Oncologia Pediatrica [Italian Association of Pediatric Hematology and Oncology]) centers. These patients were treated at 44 different AIEOP centers, with 1 center treating 62 adolescents (26% of all those treated by AIEOP centers). The remaining 142 adolescents with STS were treated at 66 non-AIEOP centers, one of which managed 17 adolescents. Centers in the north of Italy were more likely to attract patients from other regions. Conclusion: Although HDRs have some limitations, they are the only tool for investigating access to care in countries without national cancer registries. Our findings support the use of HDRs for such purposes, confirm the efficacy of the Italian pediatric oncology network, and make the case for closer collaboration between pediatric and adult oncologists.

11.
Nature ; 574(7780): 707-711, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31664194

RESUMO

In cancer, recurrent somatic single-nucleotide variants-which are rare in most paediatric cancers-are confined largely to protein-coding genes1-3. Here we report highly recurrent hotspot mutations (r.3A>G) of U1 spliceosomal small nuclear RNAs (snRNAs) in about 50% of Sonic hedgehog (SHH) medulloblastomas. These mutations were not present across other subgroups of medulloblastoma, and we identified these hotspot mutations in U1 snRNA in only <0.1% of 2,442 cancers, across 36 other tumour types. The mutations occur in 97% of adults (subtype SHHδ) and 25% of adolescents (subtype SHHα) with SHH medulloblastoma, but are largely absent from SHH medulloblastoma in infants. The U1 snRNA mutations occur in the 5' splice-site binding region, and snRNA-mutant tumours have significantly disrupted RNA splicing and an excess of 5' cryptic splicing events. Alternative splicing mediated by mutant U1 snRNA inactivates tumour-suppressor genes (PTCH1) and activates oncogenes (GLI2 and CCND2), and represents a target for therapy. These U1 snRNA mutations provide an example of highly recurrent and tissue-specific mutations of a non-protein-coding gene in cancer.

12.
Nature ; 2019 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-31597162

RESUMO

Recurrent somatic single nucleotide variants (SNVs) in cancer are largely confined to protein-coding genes, and are rare in most paediatric cancers1-3. Here we report highly recurrent hotspot mutations of U1 spliceosomal small nuclear RNAs (snRNAs) in ~50% of Sonic hedgehog medulloblastomas (Shh-MB), which were not present across other medulloblastoma subgroups. This U1-snRNA hotspot mutation (r.3a>g), was identified in <0.1% of 2,442 cancers across 36 other tumour types. Largely absent from infant Shh-MB, the mutation occurs in 97% of adults (Shhδ), and 25% of adolescents (Shhα). The U1-snRNA mutation occurs in the 5' splice site binding region, and snRNA mutant tumours have significantly disrupted RNA splicing with an excess of 5' cryptic splicing events. Mutant U1-snRNA-mediated alternative splicing inactivates tumour suppressor genes (PTCH1), and activates oncogenes (GLI2, CCND2), represents a novel target for therapy, and constitutes a highly recurrent and tissue-specific mutation of a non-protein coding gene in cancer.

13.
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
14.
Artigo em Inglês | MEDLINE | ID: mdl-31539288

RESUMO

Purpose: The trauma of the diagnosis of cancer during adolescence may affect the young people's spiritual sphere. Projects aiming to the global care of adolescents with cancer should consider also their spiritual needs: at our center, the dedicated multidisciplinary team of professionals includes a chaplain with a specific training. This article describes, throughout the patients' dialogs, how a chaplain can help patients to give voice to their emotions and thoughts about their sense of life and illness. Methods: From January 2016 to December 2017, 33 adolescent patients with solid tumors had from 2 to 12 one-to-one talks with the chaplain. We selected six specific clinical cases in which some important topics were discussed between the chaplain and the patients. Results: Patients' own voices were reported to describe some specific spiritual issues, particularly as regards how spirituality could sustain them in their need to continue to hope. Examples of specific questions during talks were as follows: "Why doesn't God do something?"; "What is the point of all this pain?"; "Why have I become ill? Why me?"; "What do you think will happen to me after I die?" Conclusions: This experience underlines the importance of the inclusion of the spiritual assistant within the multidisciplinary team dedicated to young people with cancer. The daily and constantly available presence of the chaplain in the ward, from the beginning of any treatment, can permit a good relationship with patients and help them to keep hope and move forward.

15.
Cancers (Basel) ; 11(9)2019 Sep 17.
Artigo em Inglês | MEDLINE | ID: mdl-31533233

RESUMO

Adolescents and young adults (AYA) with rhabdomyosarcoma (RMS) form a subgroup of patients whose optimal clinical management and access to care remain a challenge and whose survival lacks behind that of children diagnosed with histologically similar tumors. Understanding the tumor biology that differentiates children from AYA-RMS could provide critical information and drive new initiatives to improve the final outcome. MicroRNA (miRNA) and gene expression profiling (GEP) was evaluated in a RMS cohort of 49 tumor and 15 non-neoplastic tissues. miRNAs analysis identified miR-223 over-expression and miR-431 down-regulation in AYA, validated by Real-Time PCR and miRNA in situ hybridization (ISH). GEP analysis detected 793 age-correlated genes in tumors, of which 194 were anti-correlated. NOTCH2, FGFR1/2 were significantly down-modulated in AYA-RMS. miR-223 was associated with up-regulation of epithelial mesenchymal translation (EMT) and inflammatory pathways, whereas miR-431 was correlated to myogenic differentiation and muscle metabolism. GEP showed an increase in genes associated with CD4 memory resting cells and a decrease in genes associated with γδ T-cells in AYA-RMS. Immunohistochemistry (IHC) analysis demonstrated an increase of infiltrated CD4, CD8, and neutrophils in AYA-RMS tumors. Our results show that aggressiveness of AYA-RMS could be explained by differences in microenvironmental signal modulation mediated by tumor cells, suggesting a fundamental role of immune contexture in AYA-RMS development.

16.
J Neurooncol ; 145(1): 177-184, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31522324

RESUMO

INTRODUCTION: Diffuse intrinsic pontine glioma (DIPG) is a rare clinically, neuro-radiologically, and molecularly defined malignancy of the brainstem with a median overall survival of approximately 11 months. Our aim is to evaluate the current tendency for its treatment in Europe in order to develop (inter)national consensus guidelines. METHODS: Healthcare professionals specialized in DIPG were asked to fill in an online survey with questions regarding usual treatment strategies at diagnosis and at disease progression in their countries and/or their centers, respectively. RESULTS: Seventy-four healthcare professionals responded to the survey, of which 87.8% were pediatric oncologists. Only 13.5% of the respondents biopsy all of their patients, 41.9% biopsy their patients infrequently. More than half of the respondents (54.1%) treated their patients with radiotherapy only at diagnosis, whereas 44.6% preferred radiotherapy combined with chemotherapy. When the disease progresses, treatment strategies became even more diverse, and the tendency for no treatment increased from 1.4% at diagnosis to 77.0% after second progression. 36.5% of the healthcare professionals treat children younger than 3 years differently than older children at diagnosis. This percentage decreased, when the disease progresses. Most of the participants (51.4%) included less than 25% of their patients in clinical trials. CONCLUSION: This survey demonstrates a large heterogeneity of treatment regimens, especially at disease progression. We emphasize the need for international consensus guidelines for the treatment of DIPG, possible by more collaborative clinical trials.

17.
Childs Nerv Syst ; 35(12): 2279-2287, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31414168

RESUMO

Medulloblastoma and central nervous system (CNS) germ cell tumors are very rare in adults, while they account for 25% and 5% of brain tumors in children, respectively (Pastore et al. Eur J Cancer 42:2064-208, 2006). Pediatric experiences, mostly from randomized and controlled clinical trials, have led to different tailored treatments, based on various risk factors, including histology, and extent of disease. For medulloblastoma, biological features have recently emerged that enable therapies to be scaled down in some cases, or pursued more aggressively in the event of chromosomal and/or genetic alterations (Massimino et al. Crit Rev Oncol Hematol 105:35-51, 2016). Such refinements are still impossible for adult patients due to the lack of similar clinical trials that might provide the same or a different understanding regarding patients' prognosis, long-term survival, quality of life, and acute and late toxicities. This review aims to contribute to the debate on the treatment of adults with these two diseases and promote the creation of broad-based, national and international trials to advance our knowledge in this area and to share the skills between pediatric and adult oncologists as adolescent and young adults (AYA) brain tumor national boards are currently requiring.

18.
Artigo em Inglês | MEDLINE | ID: mdl-31449499

RESUMO

This pilot study aimed to investigate dietary changes introduced after being diagnosed with cancer in a sample of 60 consecutive children and adolescents on the basis of a self-report questionnaire. The study showed that 53% of patients changed their diet, usually "to combat the tumor" (in 47% of cases). In 1 in 2 of these cases, the health care staff were not informed about any changes. This study underscores the fact that many patients and families chose to impose dietary changes that are not always discussed with their health care staff and that may risk interfering with the continuity of a patient's treatment. It is essential to have more opportunities to speak with patients and families about this issue.

19.
J Med Humanit ; 40(4): 505-511, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31165370

RESUMO

The present paper describes the story of the development of a graphic novel-a story about superheroes-written by adolescent cancer patients on the Youth Project at the Istituto Nazionale Tumori in Milan (Italy). Nineteen patients from fifteen to twenty-five years old (fifteen receiving treatment for their cancer, four who had recently completed their treatments) participated in a four month creative writing laboratory managed by a professional teacher. The output from the writing laboratory was a written text that was used as the script for a graphic novel drawn by professional cartoonists and working together with the patients. Through their story, adolescent patients succeeded in voicing their dreams and fears. It was the adolescent patients themselves who explained how they had each tried to bring out the superhero inside them. This project describes the amazing powers of adolescents with cancer and opens a precious window on their inner world, enabling us to gain a better understanding of what they are really thinking and feeling through their own words.

20.
Med Oncol ; 36(7): 59, 2019 May 18.
Artigo em Inglês | MEDLINE | ID: mdl-31104202

RESUMO

Rhabdomyosarcoma (RMS) is rare in adults and it is generally characterized by poor outcome. In a previous retrospective study, we demonstrated a better prognosis in adults treated with multimodality approach resembling pediatric protocols. Thereafter, we developed specific recommendations based on the principles adopted in pediatric oncology. The present analysis reports the results in a subsequent prospective series. The study included 95 consecutive patients (age 18-77 years) treated from 2002 to 2015 for embryonal and alveolar RMS. As in the previous series, patients were stratified by the appropriateness of their treatment according to therapeutic guidelines for childhood RMS. The 5-year event-free survival (EFS) and overall survival (OS) rates were 33.6% and 40.3%, respectively. The 5-year EFS was 40.8% for patients with the highest treatment score, and 15% for those with lower score, while OS was 44.4% and 24.5%, respectively. The developing of specific recommendations enabled an increase in the number of patients treated with intensive multimodal treatment resembling pediatric strategy (69.7% vs. 39.1% in the retrospective series). This study reinforced the idea that adherence to the principles of pediatric protocols, improves adult RMS outcomes. However, treating adults with pediatric-type strategy is not enough to achieve the results obtained in children. Issues in compliance and a more aggressive biology of adult RMS might have a role in the different outcome according to age. Improving the collaboration between pediatric and adult oncologists in promoting specific clinical and biological research is crucial to improve the outcome for this patient population.


Assuntos
Rabdomiossarcoma/terapia , Adulto , Fatores Etários , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Terapia Combinada , Dactinomicina/administração & dosagem , Intervalo Livre de Doença , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Feminino , Humanos , Ifosfamida/administração & dosagem , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Rabdomiossarcoma/tratamento farmacológico , Rabdomiossarcoma/radioterapia , Rabdomiossarcoma/cirurgia , Taxa de Sobrevida , Vincristina/administração & dosagem , Adulto Jovem
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