Definition

Medulloblastoma is a very poorly-differentiated tumor that arises from neuroectodermal cells. It’s so poorly-differentiated, in fact, that it shows no similarity to any normal, mature CNS cell.

Incidence

This tumor is not super common overall. It most frequently occurs in children; however, it can occur in adults as well.  

Gross appearance

Unlike many other CNS tumors, medulloblastoma occurs basically in one spot: the cerebellum. In children, it occurs in the midline, but in adults it sometimes occurs in lateral regions. If the tumor grows fast enough, it can occlude the flow of CSF, causing hydrocephalus. It’s usually well-circumscribed, and it has a propensity to extend up to the surface of the cerebellum and into the subarachnoid space (and hence the CSF), where it may spread to distant sites within the CNS. Sometimes, it metastasizes to the cauda equina (these are called “drop” metastases).

Morphology

Histologically, medulloblastoma consists of sheets of tightly-packed, small, dark cells, which often have an elongated or crescent-like shape. The cells are really primitive-looking; they There are usually lots of mitoses. Sometimes the cells form little rosettes called Homer-Wright rosettes (which are also seen in neuroblastoma). They aren’t really true rosettes, if you want to be super accurate, because they don’t have a lumen (they just consist of cells surrounding a central region of neuropil). Compare these to the little rosettes (these are also technically pseudorosettes) we saw in ependymoma!

Prognosis

Without therapy, as you’d expect, prognosis is dismal. However, the silver lining is that this is one of a few malignancies that is happily, equisitely radiosensitive (another one is seminoma). With surgery and radiation, 5 year prognosis is around 75%.