ABSTRACT
Introduction: Posterior fossa ependymomas in children
are associated with a poor prognosis. This retrospective study tries to
determine prognostic factors for the outcomes and the quality of life.
Material and Methods : Thirty-three patients with
posterior fossa ependymomas were treated from 2002 to 2018. All patients
had a cranio-spinal MRI and 3.12% were metastatic. Removal was complete
in 29 patients (90.62%). All patients received a complementary
treatment: 11patients chemotherapy, radiotherapy in 35 patients, 24
cases of neoadjuvant type and for recurrence in 11.
Results : Average age was 5.8 years with a range from 9
months to 18 years. The sex ratio was 2.3 (M/F: 23/10). Sixteen patients
had one recurrence (3 metastatic). 90% of the EpPCF were of the PFA
group. Overall Survival was 65% with a mean follow-up of 8.4 and a
median of 9 years. Twenty-one patients were alive (63.63%) at last
follow-up. All were tumour-free according the MRI except 2 patients, 1
with a stable residue for 5 years and 1 in palliative treatment for
metastatic recurrence for 5 years. Post-operative complications were as
follow, facial nerve palsy in four cases (12.12%), swallowing disorders
in three cases (9.09%), transient cerebellar syndrome in 4 cases
(12.12%). Fifteen patients had normal schooling.
Conclusion : Posterior fossa ependymomas are aggressive
tumors. Complete surgical removal remains the most important prognostic
factor even if responsible of sequels. Despite molecular studies and the
expression of different genes, no obvious therapeutic target has yet
emerged.
Introduction :
Posterior fossa ependymoma (EpPCF) in children have a different behavior
from other localizations [1, 2] and differ from adult EpPCF [3,
4]. A complete removal combined with craniospinal irradiation +/-
chemotherapy is the treatment of choice [5, 6]. However, despite new
therapeutic strategies, their management remains a challenge with a high
rate of recurrence, mortality and a limited overall survival. We
reviewed our patients treated from 2002 to 2018 for a posterior fossa
ependymoma to report their evolution after surgery and new therapeutic
strategies adopted in recent years.