This case presentation describes the treatment of a 6-year-old male child diagnosed with Grade II ependymoma of the posterior fossa. He underwent suboccipital craniotomy and tumor resection, with residual tumor found on postoperative MRI. He was then referred for postoperative radiotherapy. He received 54Gy in 30 fractions to the tumor bed over 6 weeks. Follow-up showed no evidence of disease recurrence.

1. CASE PRESENTATION
Dr. Abhilash G
JR-2 Radiotherapy
SRMSIMS

2. 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.

3. 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

5.  Patient underwent Sub occipital Craniotomy
and Tumor resection on 26/03/14 at SRMS
and the histopathology revealed
Ependymoma Grade II.

8. 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.

9. PRE RADIOTHERAPY WORKUP
 Routine blood investigations – CBC, LFT, KFT
 Systemic Examination – WNL
 CECT HEAD (RTP) 18/04/2014
 3T MRI Brain 18/04/2014

12.  Spinal screening showed no deposits in the
whole spine and CSF cytology was negative
for malignant cells.

13. RADIOTHERAPY
TREATMENT PLANNING

14. CT MRI FUSION

15. CTV – SMALL RESIDUAL TUMOR

16. RECONSTRUCTION OF BODY CONTOUR AND RESIDUAL TUMOR

17. PLACEMENT OF BEAMS

18. DOSE DISTRIBUTION LIMITED TO AREA OF CONCERN

19. RADIOTHERAPY TREATMENT
 Patient received 54Gy in 30# (1.8Gy/#) with
6MV photons from 08/05/14 to 18/06/14.
(6 weeks)

20. FOLLOW UP
 Patient is under follow up with no evidence of
disease.

21. 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

22.  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.

23. 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.

24.  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

25. INDICATIONS OF RADIOTHERAPY
 High Grade disease
 Residual Disease
 Relapse

26.  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.

27.  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)

28. 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.

29. 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)

30. 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.

31. 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.

32. THANK YOU