It can occur anywhere within the brain and can have multiple sites of origins, with a high probability of metastasis through cerebrospinal fluid (CSF).
Symptoms depend on the location of the tumor and, thus, may vary, but they may include raised intracranial pressure, paresis, seizures, visual impairments, ataxia, and torticollis.
A standard treatment plan hasn't been established; common strategies involve chemotherapy and radiotherapy for individuals older than 3 years of age.
[1] This shakeup of the classification (expanded upon from the revised fourth edition, published in 2016) is due to an ongoing effort to better define tumors along their molecular features.
Histologically, ETMRs were recognized as separate entities named medulloepithelioma, ependymoblastoma, and embryonal tumor with abundant neuropil and true rosettes (ETANTR).
Another marker of the disease is high expression of LIN28A, which is often identified using immunohistochemistry and is useful for diagnosis, as it is very rarely seen in other brain tumor entities.