RESUMO
Langerhans cell histiocytosis (LCH) is a haematological disorder, affecting single or multiple organs, characterized by abnormal proliferation of Langerhans cells in children. Accurate tumour delineation (number of lesions, organs involved) is crucial for staging/re-staging, and follow-up (response to therapy). Conventional imaging techniques (computed tomography (CT), magnetic resonance imaging (MRI)) have been employed for initial diagnosis, staging and assessment of response to therapy focusing on the healing effect therapeutic protocols have on the disease. In this case report, whole-body positron emission tomography/computed tomography (PET/CT) was shown either to provide information on the metabolic activity of histiocytes, or identify lesions otherwise asymptomatic. It is clear that PET/CT, combining anatomic and metabolic information, provides data for accurate staging, therapeutic protocol selection and assessment of response to therapy.
Assuntos
Fluordesoxiglucose F18 , Histiocitose de Células de Langerhans , Criança , Humanos , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Tomografia Computadorizada por Raios X , Imageamento por Ressonância Magnética , Histiocitose de Células de Langerhans/diagnóstico por imagem , Tomografia por Emissão de Pósitrons , Estadiamento de Neoplasias , Compostos RadiofarmacêuticosRESUMO
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.