Objective:
To investigate the clinical features of pediatric
patients with intracranial primary non-germinomatous
germ cell tumors (NGGCT) and evaluate the
treatment outcomes and
prognostic factors of NGGCT.
Methods:
Clinical data of 40
children with NGGCT
who were treated with
radiotherapy (RT) at our department between November 2008 and June 2019 were retrospectively analyzed. Ninety percent of them received
craniospinal irradiation (CSI). All
children received
platinum-based
chemotherapy.
Survival analysis was conducted using the
Kaplan-Meier estimate. The
prognostic factors were analyzed by log-rank test.
Results:
The primary sites were
pineal gland, sellar / suprasellar region and
basal ganglia. The median
age of onset was 108 months (20-204 months). The median follow-up
time was 33 months (8-131 months), and the 3-year and 5-year overall
survival (OS) rates were 82.0%. The 3-year and 5-year
progression-free survival (PFS) rates were 78.6% and 73.0%. Univariate
analysis showed that increased
alpha-fetoprotein (AFP) ( P=0.02), age at first
diagnosis>10 years ( P=0.006),
metastasis at first
diagnosis ( P<0.001), and the pathological type (
choriocarcinoma,
yolk sac tumor and / or
embryonal carcinoma) ( P=0.036) were independent adverse
prognostic factors.
Conclusions:
Increased AFP, age>10 years at first
diagnosis,
tumor metastasis and pathological type were independent adverse
prognostic factors of NGGCT. The overall
prognosis of NGGCT
children is worse than that of their counterparts with
germinoma, and multidisciplinary intensive
therapy is needed to improve
survival.