/medulloblastoma with extensive nodularity

medulloblastoma with extensive nodularity

Rare Disease

MONDO:0016710

Also Known As

MBENcerebellar neuroblastomamedulloblastoma with extensive nodularitymedulloblastoma with extensive nodularity and advanced neuronal differentiationnodular medulloblastoma

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.