paediatric ependymoma (p.o)

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