Article Text

Download PDFPDF
Clinical presentation of childhood soft tissue sarcomas: a systematic review and meta-analysis
  1. Lorna Ni Cheallaigh1,
  2. Jo-Fen Liu2,
  3. Lorna Fern3,
  4. Paul Winyard1,
  5. David Walker4,
  6. Ashley Ball-Gamble5,
  7. Dhurgshaarna Shanmugavadivel2
  1. 1 Institute of Child Health, University College London, London, UK
  2. 2 Lifespan and Population Health, University of Nottingham, Nottingham, UK
  3. 3 Clinical Trials Unit, UCLH, London, UK
  4. 4 Children's Brain Tumour Research Centre, University of Nottingham, Nottingham, UK
  5. 5 Children's Cancer and Leukaemia Group, Leicester, UK
  1. Correspondence to Dr Dhurgshaarna Shanmugavadivel, General Paediatrics, Nottingham Children's Hospital, Nottingham NG7 2UH, UK; shaarnashan{at}doctors.org.uk

Abstract

Background Time to diagnosis (TTD) of childhood soft tissue sarcoma (STS) is significantly associated with survival. This review aims to identify pre-diagnostic symptoms/signs to inform earlier diagnosis interventions.

Methods Medline, Embase, Cochrane and Web-of-Science were searched between January 2010 and February 2021 for studies including children (<18 years) diagnosed with STS, with no language restrictions. Pooled proportions of symptoms/signs were calculated and subanalysed by tumour location and age.

Results Fifty-nine eligible studies were identified, totalling 2462 cases. The most frequent symptoms were lump/swelling (38%, 95% CI 27% to 51%), pain (6%, 95% CI 3% to 10%), cutaneous changes (4%, 95% CI 0 to 9%), localised eye swelling (3%, 95% CI 0 to 7%), cranial nerve deficits (2%, 95% CI 0 to 5%) and constitutional symptoms (2%, 95% CI 0 to 5%).

Symptoms varied by location and age. Localised eye swelling (20%, 95% CI 3% to 45%), cranial nerve deficits (14%, 95% CI 4% to 28%) and impaired visual function (6%, 95% CI 0 to 17%) were frequent in head and neck tumours. For abdomen/pelvic tumours, urinary symptoms (24%, 95% CI 5% to 15%), abdominal distension/discomfort (22%, 95% CI 4% to 47%), genital lump/swelling (16%, 95% CI 1% to 42%), constitutional symptoms (9%, 95% CI 0%] to 23%), vaginal bleeding (7%, 95%C I 0 to 21%) and bowel habit changes (6%, 95% CI 0 to 17%) were frequent.

In <5 years, consumptive coagulopathy (16%, 95% CI 0 to 48%), cutaneous changes (5%, 95% CI 0 to 40%), genital lump/swelling (4%, 95% CI 0 to 14%), reduced mobility (3%, 95% CI 0 to 11%), vaginal bleeding (2%, 95% CI 0 to 11%) and bleeding/bruising/petechiae (2%, 95% CI 0 to 20%) were frequent compared with lump/swelling, constitutional symptoms, pain and headaches which were frequent among >11 years.

Conclusions For STS, pre-diagnostic symptoms differ by age and location, highlighting the need to tailor early diagnosis interventions.

  • paediatrics
  • child health

Data availability statement

Data are available upon reasonable request. Data included in this study, including individual de-identified participant data and a data dictionary defining each field in the set, will be made available to others upon request via email, following completion of a signed data access agreement.

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Data availability statement

Data are available upon reasonable request. Data included in this study, including individual de-identified participant data and a data dictionary defining each field in the set, will be made available to others upon request via email, following completion of a signed data access agreement.

View Full Text

Footnotes

  • Twitter @ashleysgamble, @HeadSmartFellow

  • Contributors All authors had full access to all the data in the study and acknowledge final responsibility for the decision to submit for publication. LNC, J-FL and DS all have accessed and verified the data included in this study. LNC and J-FL completed the statistical analysis. LNC wrote the final draft with input from J-FL, DS, LF, PW and DW. DS is the guarantor for this study.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.