Symptom interval and treatment burden for patients with malignant central nervous system germ cell tumours.
Archives of disease in childhood
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Hayden, J., Murray, M., Bartels, U., Ajithkumar, T., Muthusamy, B., Penn, A., Calaminus, G., & et al. (2020). Symptom interval and treatment burden for patients with malignant central nervous system germ cell tumours.. Archives of disease in childhood, 105 (3), 247-252. https://doi.org/10.1136/archdischild-2019-317245
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 multi-centre ten-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 (58M:28F) 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 (0-23y). The time to diagnosis from first symptom (SI) was 0-69 months (median 3m, mean 9m). 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 CNS-GCT patients have >6m 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.
Humans, Neoplasms, Germ Cell and Embryonal, Brain Neoplasms, Treatment Outcome, Retrospective Studies, Cost of Illness, Adolescent, Child, Child, Preschool, Infant, Infant, Newborn, Early Detection of Cancer, Time-to-Treatment
External DOI: https://doi.org/10.1136/archdischild-2019-317245
This record's URL: https://www.repository.cam.ac.uk/handle/1810/297290
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