PROMISING OUTCOMES OF EXTRACRANIAL GERM CELL TUMORS IN CHILDREN &
ADOLESCENTS: LESSONS LEARNT OVER A DECADE.
Abstract
Background: The purpose of this single-centre study was to analyse the
outcomes of extracranial germ cell tumors (GCTs) in children treated on
a multi-modality regimen at a single-centre. Methods: Retrospective
study of children (<18 years) with a histopathologically
confirmed diagnosis of extracranial GCT over a period of 10 years
(January’09-December’18) treated on a uniform institution-based
protocol. All completely excised teratomas and stage I gonadal tumors
received no further therapy (low risk); Stage IV Ovarian, Stage III-IV
extragonadal GCTs received 6 cycles of chemotherapy (high risk) and the
remaining received 4 cycles of chemotherapy (intermediate risk).
Results: A total of 336 kids were treated of which the analysable cohort
comprised of 297with a boy-girl ratio of 1.72:1 and median age of 4
years. Gonadal GCTs(n-180) were commoner than extragonadal GCTs(n-117)
with ovary as primary site in 128 children(43%) and sacrococcygeal site
being the commonest extragonadal location(n-41;14%). LR, IR and HR
disease were noted in 60(20.2%) patients, 125(42%)patients and
112(37.8%)patients respectively. Forty-one patients relapsed and 43
children expired (disease related-33; toxic deaths-9; unknown-1). The
5-year EFS/OS was 79.3%/84.4% respectively with gonadal site, low-risk
and non-metastatic disease associated with statistically better EFS
(median follow-up:52.1±37.3 months). Conclusion(s): Both cisplatin and
carboplatin based regimens had comparable outcomes. The low and
intermediate GCTs had an excellent outcome, thus warranting a gradual
shift in the approach to these tumors by reducing therapy and decreasing
late effects of therapy. In high risk GCTs however, intensifying
therapies to improve outcomes must be balanced against the risk of
cumulative toxicity.