RESUMO
The 5-year overall survival of children and adolescents with osteosarcoma has been in plateau during the last 30 years. The present systematic review (1976-2023) and meta-analysis aimed to explore factors implicated in the prognosis of children and young adults with high-grade osteosarcoma. Original studies including patients ≤30 years and the Nationwide Registry for Childhood Hematological Malignancies and Solid Tumors (NARECHEM-ST) data (2010-2021) referred to children ≤14 years were analysed. Individual participant data (IPD) and summary estimates were used to assess the n-year survival rates, as well as the association of risk factors with overall survival (OS) and event-free survival (EFS). IPD and the n-year survival rates were pooled using Kaplan-Meier and Cox regression models, and random effects models, respectively. Data from 8412 patients, including 46 publications, NARECHEM-ST data, and 277 IPD from 10 studies were analysed. The summary 5-year OS rate was 64% [95% confidence interval (95%CI): 62%-66%, 37 studies, 6661 patients] and the EFS was 52% (95%CI: 49%-56%, 30 studies, 5010 patients). The survival rates generally differed in the pre-specified subgroups. Limb-salvage surgery showed a higher 5-year OS rate (69%) versus amputation (47%). Good responders had higher OS rates at 3 years (94%) and 5 years (81%), compared to poor responders at 3 years (66%), and 5 years (56%). Patients with metastatic disease had a higher risk of death [Hazard Ratio (HR): 3.60, 95%CI: 2.52, 5.15, 11 studies]. Sex did not have an impact on EFS (HR females/males: 0.90, 95%CI: 0.54, 1.48, 3 studies), whereas age>18 years seems to adversely affect EFS (HR 18+/<10 years: 1.36, 95%CI: 1.09, 1.86, 3 studies). Our results summarize the collective experience on prognostic factors of high-grade osteosarcoma among children and young adults. Poor response to neoadjuvant chemotherapy and metastatic disease at diagnosis were confirmed as primary risk factors of poor outcome. International collaboration of osteosarcoma study groups is essential to improve survival.
Assuntos
Neoplasias Ósseas , Osteossarcoma , Sistema de Registros , Humanos , Osteossarcoma/patologia , Osteossarcoma/epidemiologia , Osteossarcoma/mortalidade , Osteossarcoma/terapia , Criança , Prognóstico , Adolescente , Neoplasias Ósseas/epidemiologia , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Neoplasias Ósseas/terapia , Adulto Jovem , Grécia/epidemiologia , Taxa de Sobrevida , Feminino , Masculino , Pré-Escolar , Adulto , Fatores de RiscoRESUMO
BACKGROUND: Radiation therapy is stressful for both the patient and family. After a child's cancer diagnosis, parents face the burden of dealing with fear, their children's needs and the unfamiliarity of radiotherapy procedure. INTRODUCTION: This paper aims to present methods to alleviate the total psychological stress those children and parents feel during the radiation course. METHODS: A literature search was performed until January 2020. RESULT: Previous publications suggest a multidisciplinary proactive approach involving health professionals, patient, family, and extended family to lessen the stress associated with radiation therapy. A well-trained, specialist, healthcare radiotherapy-pediatric staff can help, educate and communicate effectively with the family. Maintaining the same health staff during radiotherapy creates intimacy and reduces anxiety. All necessary age-appropriate information as well as a list available with the most important things about the care-giving should be clearly written and easily accessible. An information exchange system among all the health specialists involved must be developed. Friendly and playful specialists are crucial in familiarizing the child with the computed tomography (CT) machines and radiotherapy process. A tablet with an educating application or machine models installed in the waiting area can be useful. Creative arts therapy may be a positive intervention. Maintaining a normal routine is vital for the psychological well-being of parents and children. A social worker may assist in regaining a good mental state. CONCLUSION: With ongoing age-appropriate information and psychological support throughout the whole radiation period, both parents and children will be more optimistic, strong and encouraged to fight for the child's and family's well-being.
Assuntos
Pais , Estresse Psicológico , Ansiedade , Criança , Pessoal de Saúde , Humanos , Estresse Psicológico/prevenção & controleRESUMO
PURPOSE/OBJECTIVE: About 20% of children with solid tumours (ST) present with distant metastases (DM). Evidence regarding the use of radical radiotherapy of these DM is sparse and open for personal interpretation. The aim of this survey was to review European protocols and to map current practice regarding the irradiation of DM across SIOPE-affiliated countries. MATERIALS/METHODS: Radiotherapy guidelines for metastatic sites (bone, brain, distant lymph nodes, lung and liver) in eight European protocols for rhabdomyosarcoma, non-rhabdomyosarcoma soft-tissue sarcoma, Ewing sarcoma, neuroblastoma and renal tumours were reviewed. SIOPE centres irradiating ≥50 children annually were invited to participate in an online survey. RESULTS: Radiotherapy to at least one metastatic site was recommended in all protocols, except for high-risk neuroblastoma. Per protocol, dose prescription varied per site, and information on delineation and treatment planning/delivery was generally missing. Between July and September 2019, 20/27 centres completed the survey. Around 14% of patients were deemed to have DM from ST at diagnosis, of which half were treated with curative intent. A clear cut-off for a maximum number of DM was not used in half of the centres. Regardless of the tumour type and site, conventional radiotherapy regimens were most commonly used to treat DM. When stereotactic radiotherapy was used, a wide range of fractionation regimens were applied. CONCLUSION: Current radiotherapy guidelines for DM do not allow a consistent approach in a multicentre setting. Prospective (randomised) trials are needed to define the role of radical irradiation of DM from paediatric ST.