Understanding Ependymoma: Diagnosis and Treatment for Patients

Ependymoma treatment in India

The choice of treatment used to deal with ependymoma varies according to the location of the tumor (brain or spinal cord), its size, grade, the age, and the health of the patient. That is a clear breakdown:

Typical Treatment Methods of Ependymoma

Surgery (Neurosurgical Resection)

• Initial therapy in the majority of epndymomas.
• Goal: Maximal safe removal (lose as little functionality of the brain or the spinal cord as possible to the tumor).
• En bloc [complete] resection provides the greatest likelihood of long-term control.
• A complete resection is not always achievable because of the location of such tumors.

Radiation Therapy

Host is usually advised after surgery particularly when:

• The tumor is graded as high (anaplastic ependymoma).
• Total resection could not be performed.

Types:

• Conformal radiotherapy / IMRT (Intensity-Modulated Radiation Therapy) -Tumor is precisely targeted.
• Proton beam radiation therapy - reduces damage caused to the adjacent tissues (also preferred in cases of children).
• Small, recurrent tumor may be treated by stereotactic radiosurgery (SRS).

Chemotherapy

• Not as effective in first line treatment as compared to surgery and radiation.

It is sometimes regarded as:

• Very young children (to postpone radiation).
• Recurrent and resistant ependymomas
• drugs involved might include platinum based (cisplatin, carboplatin) etoposide, cyclophosphamide, temozolomide.

Trial- Driven Therapy

• Ongoing research, some with published data, into molecular subgroups of ependymoma (e.g., RELA fusion+, posterior fossa A/B)
• Trials are under assessment.
• Immunotherapy
• Innovative (new) targeted drugs (e.g., inhibitors of the VEGF, epigenetics).
• Recommended in the case of relapsed/refractory.

Supportive & Rehabilitation Care

• Cerebral care, physical therapy, occupational therapy.
• Treatment of seizures (in case of seizures).
• Patient and family support on the psychological level.

Best hospitals for ependymoma in India

• Artemis Hospital, Gurgaon
• Medanta-The Medicity, Gurgaon
• Fortis Memorial Research Institute, Gurgaon
• Max Hospital, Saket

Factors Affecting Cost of ependymoma surgery in India

To get a better idea of what goes into the price of ependymoma surgery in India, using general brain tumor surgery statistics can be helpful--in the absence of figures strictly on ependymoma, these all-inclusive measures can be representative:

Tumor-Specific Factors

• Type, Size and Location of Tumor: Deep-seated or complex tumors (e.g. those near critical brain structures) necessitate complicated surgical procedures and an increased operating time shooting up costs.

The Hospital & Geographical Location

• Type of Hospital: Premium level of the hospital- Most private hospitals are very expensive; particularly those accredited by NABH or JCI.

• City/Tier: Higher prices can be seen in metros such as Delhi, Mumbai and Hyderabad.

Surgeon Expertise

• Experience and Reputation of the Surgeon: A high reputation of the neurosurgeon can make him demand premium rates, and this will affect price.

Diagnosis and pre-surgery investigations

The expenses of necessities tests and imaging:

• Pre-operation tests (e.g. MRI + CT scans), PET scans or advanced imaging to facilitate surgical planning incur additional cost.

Hospitalization & Postoperative Treatment

• Length of Stay & Rehab: A lengthy hospital stay and physio, neuro-rehab can significantly increase the total cost of the bill.

Pediatric ependymoma treatment in India

A concise description of how ependymoma in children is treated (it differs little between adults), and it is necessary to take into account the age, tumor biology, and long-term side effects:

Pediatric Ependymoma Main Treatment Approaches

Surgery (First line therapy)

• Goal: Curative resection with the greatest possible safety (complete removal of the tumor).
• The best curative chances are obtained by complete removal.
• Partial removal may be undertaken on exceptional grounds due to propinquity to structures that may be of importance to the brainstem.

Radiation Therapy

• An important component in the postoperative management of most children (age = 3 years).
• It is conformal radiation therapy (eg, IMRT) or proton beam therapy.
• Proton therapy is also preferable when treating children because it avoids damage to healthy parts of the brain, and the risk of subsequent problems in development, learning and endocrine functioning are minimized.
• Generally offered to tumor bed alone (not to the entire brain/ spine unless spread is known).

Chemotherapy

• Inadequate role as compared to surgery and radiation.

It is mostly applied when:

• The child is younger than <3 years old (to postpone or prevent the radiation to avoid the impact of brain development).
• Tumor recurrence or limited or incomplete resection
• Popular medicines: cis-platin, carboplatin, etoposide, vincristine, cyclophosphamide, temozolomide.

Targeted Therapy & Clinical Trials

• There are studies underway on molecular subgroups of ependymomas (e.g. RELA fusion+, posterior fossa A/B).

Clinical trials on new treatments:

• Strictly targeted drugs (e.g., VEGF inhibitors, epigenetic drugs).
• Immunotherapy approaches.
• It is usual to encourage children to participate in clinical trials where available.

Supportive and Rehab Care

• Neurocognitive rehabilitation (speech, memory, school support).
• Physical/occupational training of movements.
• Management of seizure, in the event it happens.
• Endocrine follow-up (e.g. thyroid, growth and puberty problems caused by radiation).
• Mental care of child/family.

Advanced therapies for ependymoma in India

A logical outline of the sophisticated and novel strategies against ependymoma is described, beyond the conventional surgery and radiation treatment:

Accurate Radiation Therapies

PBT (Proton Beam Therapy):

• More accurate than the traditional photon-based radiation.
• Minimizes healthy brain/spinal radiation exposure → fewer long term effects, where children are most at risk.

SRS (Stereotactic Radiosurgery):

• High dose radiation targeted (Gamma Knife, CyberKnife).
• Applicable in case of small tumors or in recurring tumors where there is no possibility of repeat operation.

MRT (Intensity - Modulated Radiation Therapy):

• Transfers radiation dose in shaped amounts in order to minimize collateral injuries.

Novel Drug Combinations Chemotherapy

Conventional chemo is low on efficacy but combination therapies at an advanced stage are under trial:

• Platinum containing (cisplatin, carboplatin)
• Vincristine, the cyclophosphamide, and Etoposide
• Temozolomide (oral, is sometimes used in case of recurrence).
• New metronomic chemotherapy regimens (low-dose continuous) that have been investigated to control recurrences.

Targeted & Molecular Therapies

The new research is moving towards ependymoma molecular subtyping as a direction to the therapies:

• Supratentorial ependymomas RELA fusion- identified
• Difference between the A and B subtypes of posterior fossa

Targets/drugs under development:

• Angiogenesis inhibitors (such as bevacizumab), to inhibits tumor blood vessel formation.
• TOR/ PI3K pathway inhibitors - on tumors with activation of the pathway.
• HDAC inhibitors, EZH2 inhibitors.

Immunotherapy Approaches

Cancer Vaccines:

• Peptide or dendritic-cell immunization directed against antigens which are PD specific.
• Examples: peptide vaccine trials that target RELA fusion proteins.

On Immune Checkpoint Inhibitors (ICIs):

• PD-1/PD-L1 and CTLA-4 monoclonal antibody agents are under investigation in high grade and recurrent tumors.

Oncolytic Viruses:

• Genetically engineered viruses that can infect and kill only tumour cells and activate immunoreactions.

Personalised and genomics-based care

• Tumor genome sequencing to provide options to cure one by one mutation.
• Liquid biopsy (circulating tumor DNA) is an area of interest in non-invasive monitoring.

Re-opening the treatment & Combination Therapies

• Indications In recurrent ependymomas, re-irradiation with sophisticated modalities (proton therapy, stereotactic radiosurgery) is promising.
• Combinations with radiation and immunotherapy is under testing, as well as chemo and targeted therapy.

Clinical Trials

Several worldwide clinical trials are on-going in pediatric and adult ependymomas:

• Other clinical trials on immunotherapy (checkpoint inhibitors, vaccines).
• Trials involving HDAC inhibition, PARP inhibition and VEGF blockage.
• Pediatric studies with an emphasis on allaying late side effects but ensuring improved survival.

Top neurosurgeons for ependymoma in India

• Dr. Aditya Gupta
• Dr. Sudhir Dubey
• Dr. V P Singh
• Dr. Rana Patir
• Dr. Arun Saroha

Ependymoma treatment in India by GetWellGo

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