Medulloblastoma with extensive nodularity
ORPHA:251858· ICD-10 C71.6
Definition
Medulloblastoma with extensive nodularity (MBEN) is a histological variant of medulloblastoma, an embryonic malignancy, most often located in the inferior medullary velum and then growing into the fourth ventricle, and presenting in infants and young children with symptoms of increased intracranial pressure such as headache, listlessness, vomiting, diplopia and papilledema. It is often associated with Gorlin syndrome and has a relatively good prognosis.
- Inheritance
- Not applicable
- Age of onset
- Childhood, Infancy