From the Guidelines
The primary treatment for ependymoma is surgical removal of the tumor, with the goal of achieving complete resection when possible, as recommended by the most recent guidelines 1.
Treatment Overview
The treatment approach for ependymoma involves a combination of surgery, radiation therapy, and in some cases, chemotherapy.
- Surgery is the initial treatment, aiming for gross total resection whenever feasible, especially for intracranial WHO grades II and III ependymomas in adults 1.
- Postoperative radiation therapy is recommended, particularly for higher-grade tumors (WHO grade III) or in cases of incomplete resection, with doses ranging from 54-59.4 Gy 1.
- Chemotherapy may be considered for young children to delay radiation or for recurrent disease, though its effectiveness is less established than surgery and radiation 1.
Radiation Therapy
Radiation therapy plays a crucial role in the treatment of ependymoma, especially for higher-grade tumors or when complete surgical resection is not possible.
- The radiation dose typically ranges from 54-59.4 Gy, delivered over 6 weeks to the tumor bed with a 1-2 cm margin 1.
- For cases with CSF or spinal dissemination, craniospinal irradiation (CSI) of 36 Gy is recommended, with a boost up to 45-54 Gy on focal lesions 1.
Chemotherapy
Chemotherapy is used in specific situations, such as delaying radiation in young children or treating recurrent disease.
- Common chemotherapy regimens include combinations of cisplatin, carboplatin, cyclophosphamide, etoposide, and vincristine 1.
- The choice of chemotherapeutic drugs depends on previous exposures, and participation in clinical trials should be explored 1.
Follow-Up
Regular follow-up imaging (MRI) is essential to monitor for recurrence, typically every 3-4 months initially, then less frequently over time 1.
- Long-term follow-up is necessary due to the risk of asymptomatic and/or late relapses 1.
From the Research
Definition of Ependymoma
- Ependymoma is a type of glial tumor that arises from the ependymal cells lining the ventricles of the brain and spinal cord canal 2.
- It can occur in the brain or spinal cord, with spinal ependymomas being less frequent and exhibiting a better prognosis than intracranial ependymomas 3.
Treatment Options for Ependymoma
- The standard treatment for ependymoma typically involves maximal surgical resection followed by focal irradiation, except in cases of disseminated disease 2.
- Chemotherapy has not played a significant role in most treatment regimens for ependymoma, but it is being reinvestigated in ongoing studies, particularly for patients with unresectable, macroscopic, metastatic, or anaplastic disease 2.
- Conformal radiotherapy is recommended for grade 3 or incompletely resected grade II tumors, while proton therapy is increasingly employed, especially in children, to reduce the risk of neurocognitive and endocrine sequelae 3.
- Craniospinal irradiation is reserved for metastatic disease, and chemotherapy is commonly used as salvage treatment for patients failing surgery and radiotherapy 3.
Emerging Novel Therapies
- Next-generation therapies, including tyrosine kinase inhibitors, telomerase inhibitors, anti-angiogenesis agents, and immunotherapy, are being explored to address the limitations of current therapies, such as relapse, radiation-induced cognitive deficits, and chemotherapy resistance 4.
- The discovery of new druggable pathways may increase the therapeutic armamentarium for ependymoma in the near future 3.
Outcomes and Prognosis
- The extent of resection has a significant impact on outcomes, with complete resection being associated with better prognosis 3.
- The 4-year progression-free survival rate for patients with ependymoma has been reported to be around 22%, and the overall survival rate has been reported to be around 59% 5.
- Supratentorial tumor location and complete surgery have been identified as favorable factors associated with better outcomes 5.