Medulloblastoma with extensive nodularity

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Medulloblastoma with extensive nodularity

ORPHA:251858Histopathological subtype

Also called MBEN

What it is

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.

Key facts

Age of onset
Childhood, Infancy
Inheritance
Not applicable
Classified as
Histopathological subtype

Recorded for the broader condition

Prevalence
1-9 / 100 000 (Europe)Medulloblastoma

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Gene

SUFUMajor susceptibility factor

ICD-10 codes

C71.6filed under a broader ICD-10 category — shared with 4 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0016710OMIM 155255UMLS C1334970

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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