Ependymomas of the adult: molecular biology and treatment

To review state of art and relevant advances in the molecular biology and management of ependymomas of the adult.

Ependymomas of the adult are uncommon neoplasms of the central nervous system, and may occur either in the brain or the spinal cord. Compared with intracranial ependymomas, spinal ependymomas are less frequent and exhibit a better prognosis. Studies performed on genetic changes in ependymoma provide some insight into the pathogenesis and prognostic markers and yield new therapeutic targets, particularly focused on signal transduction modulators. The majority of studies have shown a major impact of extent of resection; thus, a complete resection must be performed, whenever possible, at first surgery or at reoperation. Involved field radiotherapy is recommended for anaplastic or incompletely resected grade II tumors. Craniospinal irradiation is reserved for metastatic disease. Chemotherapy is not advocated as primary treatment, and is best utilized as salvage treatment for patients failing surgery and radiotherapy.

Owing to the rarity of the disease, the literature regarding ependymomas in adults is scarce and limited to retrospective series. Thus, the level of evidence regarding therapeutic strategies is low and universally accepted guidelines are lacking. Molecular biology studies suggest some potential new therapeutic targets.