From the Guidelines
Ependymoma of the brainstem is a rare and challenging tumor that requires prompt diagnosis and treatment to minimize morbidity and mortality, with surgery being the initial treatment of choice, followed by radiation therapy in cases of incomplete resection or high-grade tumors, as recommended by the most recent guidelines 1.
Diagnosis and Treatment
The diagnosis of ependymoma of the brainstem involves craniospinal MRI and histological assessment of tissue specimens according to the World Health Organization (WHO) classification 2016 1. Treatment strategies are based on the tumor's grade and the patient's age, with gross total resection being the main goal of surgery, and postoperative conformal radiotherapy recommended for patients with WHO grade III ependymomas or incomplete resection of WHO grade II ependymomas 1.
Key Considerations
- Symptoms of brainstem ependymoma may include headaches, nausea, vomiting, balance problems, facial weakness, difficulty swallowing, and limb weakness or sensory changes.
- The prognosis depends on several factors, including the extent of surgical removal, the tumor's grade, and the patient's age, with children generally having better outcomes than adults.
- Regular follow-up imaging is essential to monitor for potential recurrence after treatment.
Treatment Recommendations
- Surgery is the initial treatment of choice, with the goal of achieving a gross total resection whenever feasible 1.
- Postoperative conformal radiotherapy is recommended for patients with WHO grade III ependymomas or incomplete resection of WHO grade II ependymomas 1.
- Chemotherapy may be used in certain cases, particularly for young children to delay radiation, or for recurrent tumors 1.
From the Research
Definition and Classification of Ependymoma
- Ependymomas are rare primary central nervous system tumors that can form anywhere along the neuraxis 2, 3.
- They are classified as grade I, II (classic), or III (anaplastic) based on the WHO 2016 classification 2.
- Ependymal tumors represent a heterogeneous group of gliomas, and recent advances in molecular characterization have identified nine molecular subgroups of ependymal tumors 2, 3.
Location and Treatment of Ependymoma
- Ependymomas can occur in the brain's supratentorial region, posterior fossa, or in the spinal cord 3, 4.
- Surgery is the primary initial treatment for ependymomas, while radiotherapy is employed as an adjuvant treatment in some cases of grade II and in all cases of anaplastic ependymoma 2, 5.
- Chemotherapy is reserved for recurrent cases, and new targeted agents or immunotherapies are being explored for ependymoma patients 2, 3.
Brainstem Ependymoma
- While the provided studies do not specifically focus on brainstem ependymoma, they do mention that ependymomas can occur in the brain's posterior fossa, which includes the brainstem 3, 4.
- The treatment and management of brainstem ependymoma would likely follow the same principles as other intracranial ependymomas, with surgery and radiotherapy playing important roles 2, 5.