Posterior fossa ependymomas in children: still a challenge for pediatric
neurosurgeons and oncologists.
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.