paediatric ependymoma (p.o)
TRANSCRIPT
CASE PRESENTATION
Dr. Abhilash G
JR-2 Radiotherapy
SRMSIMS
CASE DETAILS
Name – ABC
Age & Sex – 6 yr male child
Address – Gangapur, Bareilly
Presented in Radiotherapy OPD in April 2014
as an operated case of Ependymoma of
Posterior Fossa.
PAST HISTORY
Patient had
- Severe Headache x 1 ½ months
- Vertigo x 1 ½ months
- Nausea and Vomiting x 1 ½ months
- Constipation and Loss of Appetite
Patient underwent Sub occipital Craniotomy
and Tumor resection on 26/03/14 at SRMS
and the histopathology revealed
Ependymoma Grade II.
TREATMENT PLAN
Patient was referred to our department for
Post-op adjuvant treatment.
Patient got registered in our OPD on
29/04/14 and was planned for Post-op
Radical RT.
PRE RADIOTHERAPY WORKUP
Routine blood investigations – CBC, LFT, KFT
Systemic Examination – WNL
CECT HEAD (RTP) 18/04/2014
3T MRI Brain 18/04/2014
Spinal screening showed no deposits in the
whole spine and CSF cytology was negative
for malignant cells.
RADIOTHERAPY
TREATMENT PLANNING
CT MRI FUSION
CTV – SMALL RESIDUAL TUMOR
RECONSTRUCTION OF BODY CONTOUR AND RESIDUAL TUMOR
PLACEMENT OF BEAMS
DOSE DISTRIBUTION LIMITED TO AREA OF CONCERN
RADIOTHERAPY TREATMENT
Patient received 54Gy in 30# (1.8Gy/#) with
6MV photons from 08/05/14 to 18/06/14.
(6 weeks)
FOLLOW UP
Patient is under follow up with no evidence of
disease.
INTRODUCTION
Ependymoma accounts for only 1.8% of all adult brain
tumours.
Ependymomas arise from the ependymal cells lining the
cerebral ventricles and the vestigial central canal of the
spinal cord.
ROSETTE FORMATION – PATHOLOGICAL HALMARK
Pediatric ependymomas – Arise intracranially
Adult Ependymomas – Arise in Spinal cord (75%)
Presence of increased cellularity, cytological atypia and
micro vascular proliferation suggest anaplastic
The most frequent location is the fourth ventricle.
Tumors with ventricular involvement often cause increased
ICP, hydrocephalus, headache, nausea, vomiting,
papilledema, ataxia and vertigo.
Our patient had headache, nausea, vomiting and vertigo.
Grade II or differentiated ependymomas make up the
majority.
Presence of calcification in a fourth ventricle tumor on CT is
very suggestive of an ependymoma.
ROLE OF SURGERY
Maximal surgical resection, including second
surgery if necessary is the initial treatment of
ependyoma.
Complete resection is achieved in only 40-
60% of cases.
In our patient, there was small residual
disease in post op MRI.
Post op RT improves the recurrence free survival
of patients with intracranial ependymomas, and 5-
year survival rates with doses of 45Gy or more
range from 40% to 87%. (1)
(1). Leibel SA, Sheline GE. Radiation therapy for neoplasms of the brain. J Neurosurg 1987; 66(1):1
There appears to be a radiation dose response,
with improved tumor control with doses > 50Gy,
and doses of 54 to 59.4Gy are typically
prescribed.
ROLE OF RADIOTHERAPY
INDICATIONS OF RADIOTHERAPY
High Grade disease
Residual Disease
Relapse
Historically, for posterior fossa tumors, the
entire craniospinal axis and later the entire
posterior fossa has been irradiated.
Modern series document that local
recurrence is the primary pattern of failure.
Patients with neuraxis spread (positive MRI
or positive CSF cytology) should receive CSI
(40-45Gy) with boosts to the areas of gross
disease and to the primary tumor to total
doses of 50-54Gy.
Rogers et al studied 45 patients with
nondisseminated posterior fossa
ependymomas.
32 underwent GTR and 13 underwent STR
with or without subsequent RT.
The 10 yr actuarial local control rate was
100% for patients who underwent GTR + RT;
50% for GTR alone and 36% for those who
underwent STR and RT.
The 10 yr OS was 83% vs 67% vs 43%
(GTR + RT vs GTR alone vs STR +RT)
ROLE OF CHEMOTHERAPY
There is no evidence that chemotherapy
improves survival in these cases.
The primary application of chemotherapy is
investigational and it is within the realm of
neoadjuvant therapy to improve resectability.
NOVEL THERAPIES
Novel therapies to target molecular pathways
are currently under investigation.
Co expression of ERBB2 and ERBB4 has
been described in over 75% of pediatric
ependymoma and impact prognosis.
Antiangiogenesis drugs are also being
examined – Bevacizumab, ZD6474,
Metronomic Therapy (Thalidomide and
Celecoxib)
PROGNOSIS
Despite multimodality therapy, 50% patients
with ependymoma will experience a relapse.
Majority of recurrences are local, and
prognosis is poor after relapse.
Resection, reirradiation, and chemotherapy
are the common treatment modalities for
relapsed ependymomas.
CONCLUSION – EVIDENCE BASED SUMMARY
Maximal surgical resection should be
performed when feasible.
Postoperative radiotherapy is considered the
standard, but no prospective trials have
validated it’s role. CSI is used only in patients
with disseminated disease.
The role of chemotherapy remains to be
defined.
THANK YOU