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