Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Ependymal tumor
Synonyms
Definitions

A tumor of neurectodermal origin arising from ependymal cells that line the ventricles and central canal of the spinal cord, that can occur in both children and adults, and that is characterized by wide a range of clinical manifestations depending on the location of the tumor, such as intracranial hypertension for tumors originating in the posterior fossa, behavioural changes and pyramidal signs for supratentorial tumors, and dysesthesia for tumors of the spinal cord. They can be classified as myxopapillary ependymoma, subependymoma, ependymoma (low grade tumors) or anaplastic ependymoma (grade III tumors).

ID

http://www.orpha.net/ORDO/Orphanet_301

definition

A tumor of neurectodermal origin arising from ependymal cells that line the ventricles and central canal of the spinal cord, that can occur in both children and adults, and that is characterized by wide a range of clinical manifestations depending on the location of the tumor, such as intracranial hypertension for tumors originating in the posterior fossa, behavioural changes and pyramidal signs for supratentorial tumors, and dysesthesia for tumors of the spinal cord. They can be classified as myxopapillary ependymoma, subependymoma, ependymoma (low grade tumors) or anaplastic ependymoma (grade III tumors).

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=301

has_age_of_onset

All ages

hasDbXref

OMIM:137800

UMLS:C1333407

label

Ependymal tumor

notation

Clinical group

ORPHA:301

prefixIRI

ORDO:Orphanet_301

prefLabel

Ependymal tumor

present_in

United Kingdom AND has_annual_incidence_average_value : 0.2 AND has_annual_incidence_range : 1-9 / 1 000 000

Europe AND has_point_prevalence_range : 1-9 / 100 000

United States AND has_annual_incidence_average_value : 0.43 AND has_annual_incidence_range : 1-9 / 1 000 000

Europe AND has_lifetime_prevalence_average_value : 3.85 AND has_lifetime_prevalence_range : 1-9 / 100 000

Europe AND has_annual_incidence_average_value : 0.2 AND has_annual_incidence_range : 1-9 / 1 000 000

subClassOf

http://www.orpha.net/ORDO/Orphanet_182067

http://www.orpha.net/ORDO/Orphanet_557492

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