Clinical presentation of bone tumours in children and young people: a systematic review and meta-analysis

Abstract

Background: Children and young people (CYP) with bone tumours often experience lengthy intervals prior to diagnosis that can lead to increased morbidity and mortality. Early diagnosis is key to optimising treatment options and long-term outcomes. This review aims to describe symptomatology at diagnosis, in order to develop interventions to accelerate diagnosis.

Methods: A literature search of MEDLINE and EMBASE was conducted for studies published between January 2008 and May 2023, reporting signs and symptoms in CYP with bone tumours. Pooled proportions of symptoms and signs were calculated.

Results: 16 studies (1452 patients; 492 osteosarcoma and 932 Ewing's sarcoma) were included in the analysis. The most prevalent symptoms were pain (64%, 95% CI 52% to 75%) and swelling (22%, 95% CI 6% to 42%). Other symptoms included fever, pain and swelling, history of trauma, pathological fracture, palpable mass, functional limitation, increased volume and limp. Subanalysis identified differences in symptom clusters: osteosarcoma more frequently presented with a history of trauma, pathological fracture, pain on weight-bearing, pain worse at night, pain at rest and weight loss. Ewing's sarcoma was associated with fever, functional limitation and a palpable mass.

Conclusions: These data highlight the differences in presentations between osteosarcoma and Ewing sarcoma, which may account for differences in survival and outcome. This will be used to inform professional and public health interventions through the Child Cancer Smart campaign to accelerate diagnosis for all. This review also highlights the need for a renewed research focus to identify patients earlier in the disease development as a strategy to improve the current static outcomes.

Keywords: Child Health; Paediatrics.

Figure 1. Progress through the meta-analysis.

Figure 2. Pooled proportions for the most common prediagnostic signs and symptoms for bone tumour. (a) all cases from 16 eligible studies, (b) studies reported all bone tumour types, (c) Ewing’s sarcoma and (d) Osteosarcoma.

Figure 3. Pooled proportions for the most common prediagnostic signs and symptoms for bone tumour in children aged 0–15 years.

Figure 4. Pooled proportions for the most common prediagnostic signs and symptoms for bone tumour. (a) high-income countries (HICs), (b) low-income and middle-income countries (LMICs) and (c) comparison between HIC and LMIC.

Figure 5. Comparison of pooled proportions of common symptoms between osteosarcoma and Ewing's sarcoma.