Abstract
AIMS This study analysed the clinical features of children with
intracranial germ cell tumours (IC-GCTs) treated at two European
centres. We retrospectively reviewed timelag between symptoms onset,
clinic-radiological findings, diagnosis and outcomes. METHODS Symptoms
at diagnosis were divided into four groups: 1)raised intracranial
pressure (RICP); 2)visual impairment; 3)endocrinopathy; 4)other. Total
diagnostic interval (TDI), defined as the interval between symptom onset
(including retrospective recall of symptoms) and definitive diagnosis of
IC-GCT, was calculated and compared to survival rates. RESULTS Our
cohort included 55 children with a median follow-up of 78.9 months
(0.5-249.9). The majority (63.6%) had germinomas and 10.9% were
metastatic at diagnosis. IC-GCTs were suprasellar (41.8%), pineal
(36.4%), bifocal (12.7%) or in atypical sites (9.1%). The most common
presenting symptoms were related to RICP (43.6%); however, by the time
of tumour diagnosis, 50.9% of the patients had developed endocrine
dysfunctions. All pineal GCTs manifested with RICP or visual impairment.
All suprasellar GCTs presented with endocrinopathies. TDI ranged between
0.25-58.5 months (median 4 months). Pineal GCTs had the shortest TDI
(median TDI 1 month versus 24 months in suprasellar GCTs,
p<0.001). TDI >6 months was observed in 47.3% of
patients and was significantly associated with endocrine presenting
symptoms. No statistically significant difference was found in
progression-free survival and overall survival between patients with TDI
>6 months and with TDI 6 months. CONCLUSION Approximately
half of our patients had TDI >6 months, mostly with
endocrine deficiencies as presenting symptoms. TDI >6
months was not associated with increased relapse rate or mortality.