A retrospective analysis of recurrent pediatric ependymoma
reveals extremely poor survival ind ineffectiveness of current
treatments across central nervous locations and molecular subgroups.
Tryggve Lundar MD, PhD1,2, Bernt Johan Due-Tønnessen
MD,PhD1
Radec Fric MD,PhD1
Department of Neurosurgery, Oslo University Hospital1and University of Oslo2
Correponding author:
Tryggve Lundar
Department of Neurosurgery
Oslo University Hospital
Postboks 4950 Nydalen, Oslo, Norway
Email:
tryggve.lundar@gmail.com
Total word count: 474
Short running title: GTR can improve outcome after relapse of pediatric
ependymoma
Key words: Pediatric ependymoma, relapse, repeat surgical resection
(GTR)
Number of tables: 0
Number of figures: 0
Letter to the Editor
Pediatr Blood Cancer
Dear Editor,
RE: Ritzmann TA, Rogers HA, Paine SML, Storer LCD, Jacques TS, Chapman
RJ, Ellison D, Donson AM, Foreman NK, Grundy RG.
A retrospective analysis of recurrent pediatric ependymoma reveals
extremely poor survival ind ineffectiveness of current treatments across
central nervous locations and molecular subgroups.
Pediatr Blood Cancer 2020;67:e28426
https://doi.org/10.1002/pbc.28426
Congratulations to the authors with their detailed analysis of further
management and outcome in pediatric patients who experience recurrence
within a few years after initial treatment for ependymomas.
Initial treatment for posterior fossa ependymomas (PFE) is maximal
surgical resection (Gross total resection-GTR; if possible) followed by
local radiotherapy or chemotherapy in small children. For supratentorial
ependymomas (STE) GTR is also recommended (if possible) with or without
postoperative radiotherapy.
The management at relapses is, however, without consensus. The authors
confirm the grave prognosis for these children. In the beginning of the
discussion they state: Although primary surgery and irradiation reduced
relapse risk variability in different intracranial locations, once a
patient recurred these interventions gave, at best, short-term benefits,
confirming the need for better therapies.
This of course true – better therapies are urgently needed. They point
to the lack of consensus regarding treatment at relapse, but recent
guidelines have recommended the use of reirradiation and further
surgery. In the conclusion they underline that recurrent pediatric
ependymoma is highly aggressive with extremely poor outcome.
This negative statement, is to some extent, in conflict with the results
given under 3.5.2. At relapse: GTR at first relapse was associated with
sustained improved EFS (25% vs 0% 10-year survival).
This statement is in accord with Vinchon et al1 :
Total resection is the only curative treatment for RIE (recurrent
intracranial ependymoma) and is often possible, especially when the
initial resection was total.
How often GTR is within reach at local relapes may be a difficult
matter.
We have, however, observed several patients who underwent GTR after
recurrence, and are tumor-free today after many years of further
follow-up(up to 27 years) without any additional
treatment2. We recognize that these patients are few
compared to the majority of pediatric ependymoma patients who do not
grow-up to be well functioning adults. It is, however, important for
these small patients and their caretakers to have a hope for cure even
after relapse. The role of GTR if possible may be under-communicated.
Kind regards
Tryggve Lundar
Bernt Johan Due-Tønnessen
Radek Fric