There is no evidence that ependymoma can be cured, nor can it be completely cured. The potential for cure remains high despite a large number of patients treated with RT, albeit many of whom are undertreated.
Ependymoma is a rare type of brain tumor that typically forms in young children, affects the spinal cord and is characterized by blood vessels and glial cells. It is a benign but potentially disabling disease that may also cause death by brain obstruction. The diagnosis often requires a multimodial approach.
Signs of ependymoma in children include unexplained headache, abdominal pain or constipation. In adults, they can include neck pain, vomiting or urinary retention.\n\nOn examination, signs of ependymoma may include palpable cerebellar or spinal masses and ipsilateral pleural effusions.\n\nThe differential diagnosis for spinal mass and/or pleural effusion include lymphomas, carcinomatosis, metastatic disease or metastatic tumors from another primary tumor.\n\nThe treatment for ependymoma depends upon where the ependymoma may be located within the central nervous system (CNS).
Although we hypothesize a potential role of oncogenic genetic events such as mutations in RAS or EGFR, we found no evidence of mutations in the genes or genes involved in mitogen-activated protein kinase or the proto-oncogene pathways in ependymomas. Therefore, the possibility of genetic alterations in ependymomas is still questioned.
Almost one-half of ependymomas were surgically and/or radiologically eradicated by surgery alone or in conjunction with radiotherapy. Of the 22 % who were not completely eradicated, about 90 % underwent radiotherapy. The remainder received postoperative radiotherapy combined with chemotherapy or radiation. In general, patients whose disease was well localized could be treated using surgery alone with an excellent prognosis. However, these results are difficult to interpret because there is no evidence-based standard therapy. In addition to surgical removal and high-dose radiation therapy, we believe multimodal therapies containing chemotherapy should be encouraged in patients with well-differentiated and low-grade ependymomas with a single nonenhancing tumor.
In the U.S. population, the age-standardized incidence per 10,000 of ependymoma is 2.2 in males and 1.9 in females, and the age-standardized mortality rate is 0.6 per 10,000 of population. The incidence rate increased between 1997 and 2005. The age-standardized incidence rate has remained stable since 1996, and the age-standardized mortality rate was decreased from 1996 to 2005. These national trends suggest a decrease in ependymoma incidence from the early 1990s to 2005 but no decline in mortality.
More than 70% of patients with newly treated primary PNETs experienced disease progression. Although radiation therapy is important in controlling disease, its efficacy appears to be limited. At present, new therapeutic approaches are required that maximize disease control while limiting treatment-associated toxicity.
Irradiation resulted in a significant, but small, improvement in one domain of quality of life. In the larger group of patients with ependymoma, the greatest change was in the general physical fitness. In a recent study, findings highlight a need for larger studies to assess the impact of irradiation on quality of life.
This analysis suggests that ionizing radiation does not appear to confer any additional benefit for patients with a first complete remission of GBM versus those treated with surgery.
Most families with a diagnosis of cED have one or more members with multiple cED cases. However, there is an absence of familial cases among the sporadic cED population, supporting genetic and epigenetic models for the disease.
ependymoma is best managed by multidisciplinary treatment with surgery, chemotherapy, and radiotherapy. It can also be targeted by gene therapy, chemotherapy, or other novel treatments. Unfortunately, clinical trials for these treatments do not have any standards.
The present study suggests that a total dose of 70 Gy is more effective in the treatment of brainstem-ependymal tumors than a dosage of 30 Gy or a placebo, at least when treated early after diagnosis.