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Symptom interval and treatment burden for patients with malignant central nervous system germ cell tumours
  1. James Hayden1,2,
  2. Matthew J Murray3,
  3. Ute Bartels4,
  4. Thankamma Ajithkumar5,
  5. Brinda Muthusamy6,
  6. Anthony Penn7,8,
  7. Gabriele Calaminus9,
  8. James Nicholson10
  1. 1Department of Paediatric Haematology and Oncology, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK
  2. 2Department of Paediatric Haematology and Oncology, Great North Children’s Hospital, Newcastle upon Tyne, UK
  3. 3Department of Paediatric Haematology and Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  4. 4Department of Paediatric Haematology and Oncology, Paediatric Brain Tumour Program, SickKids, Toronto, Ontario, Canada
  5. 5Department of Radiation Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, Cambridgeshire, UK
  6. 6Department of Paediatric Neuro-Ophthalmology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  7. 7Children’s Brain Tumour Research Network (CBTRN), Royal Manchester Children's Hospital, Manchester, UK
  8. 8Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK
  9. 9Department of Paediatric Haematology and Oncology, University Hospital Bonn, Bonn, Germany
  10. 10Paediatric Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  1. Correspondence to Dr Matthew J Murray, Department of Paediatric Haematology and Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge CB2 0QQ, UK; mjm16{at}cam.ac.uk

Abstract

Objective Patients with central nervous system germ cell tumours (CNS-GCTs) commonly initially present to primary care or general paediatricians. Prolonged symptom intervals (SI) are frequently seen in CNS-GCTs and have been associated with inferior outcomes in other brain tumours. This study reviewed the clinical presentation of CNS-GCTs and examined the effect of prolonged SI.

Design/Setting/Patients/Outcomes International multicentre 10-year retrospective study (2002–2011 inclusive), across six international paediatric oncology treatment centres. All newly diagnosed patients with CNS-GCT were included. Main outcome measure was time interval from first symptom to diagnosis.

Results The study cohort included 86 (58 males:28 female) patients (59 ‘germinoma’ and 27 ‘non-germinomatous’ GCTs), with tumours being pineal (n=33), suprasellar (n=25), bifocal (pineal+suprasellar; n=24) and ‘other’ site (n=4), of which 16 (19%) were metastatic. Median age at diagnosis was 14 years (0–23 years). The time to diagnosis from first symptom (SI) was 0–69 months (median 3 months, mean 9 months). A prolonged SI (>6 months) was observed in 28/86 patients (33%) and significantly associated with metastatic disease (11/28 (39%) vs 5/58 (9%); p=0.002)) at diagnosis, but not overall survival. With prolonged SI, endocrine symptoms, particularly diabetes insipidus, were more common (21/28 (75%) vs 14/58 (24%) patients; p<0.002), but raised intracranial pressure (RICP) was less frequent (4/28 (14%) vs 43/58 (74%) patients; p<0.001)) at first symptom.

Conclusions One-third of patients with CNS-GCT have >6 months of symptoms prior to diagnosis. Delayed diagnosis is associated with metastatic disease. Early symptom recognition, particularly related to visual and hormonal disturbances in the absence of RICP, may improve timely diagnosis, reduce metastatic disease frequency and consequently reduce treatment burden and late effects.

  • brain tumour
  • symptom interval
  • prolonged symptom interval
  • Outcomes research
  • intervention
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Footnotes

  • JH and MJM are joint first authors.

  • 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.

  • Patient consent for publication Not required.

  • Ethics approval Ethical approvals were obtained according to institutional and national standards for retrospective service evaluations, requirements which varied between participating countries.

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

  • Data availability statement Data are available on reasonable request.

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