Clinical presentation and deferred diagnosis
Initial symptoms depend upon location and size of the tumour. Pineal
tumours typically cause obstructive hydrocephalus with headache,
vomiting, papilledema, and lethargy. Pineal tumours may also present
with ‘Parinaud’s syndrome’, caused by tectal plate compression. Patients
with IC-GCTs may show behavioural changes, poor school performance and
altered neurology, especially in tumours arising within the basal
ganglia. Suprasellar GCTs most commonly cause hypothalamic/pituitary
dysfunctions and may compress the optic chiasm causing visual field
defects 15, 16.
A prolonged symptom interval (i.e. the time from symptom onset to
diagnosis) has been reported in several paediatric brain tumours17–19. Reports from two institutional series have
identified a wide variation in symptom interval, with diagnosis
occurring up to 3 years from symptom onset 17,18. In
recent single-centre studies, 54% 17 and 30%15 of patients with IC-GCT had a symptom interval
>6 months. In another study 17/181 patients with IC-GCT
(9%) had a deferred diagnosis of >3 months and survival in
a small subset of germinoma patients (13/119 cases; 11%) was negatively
affected 20. Other previous studies have identified
that children with shorter symptom intervals have more aggressive
tumours, suggesting a relationship between diagnostic delay and survival21,22.
Our study evaluates retrospectively the clinical features and outcomes
of paediatric patients with IC-GCTs treated at two centres over 25
years. We reviewed the timing interval between symptoms onset,
radiological manifestations, and definitive diagnosis of IC-GCT.
METHODS