Subtype
Dysembryoplastic Neuroepithelial-Like Tumor of the Septum Pellucidum
CI-CAN-00002558Explore in graph →
Non-malignant
- NCIt
- C180532
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Subtype
CI-CAN-00002558Explore in graph →
Data completeness0 / 7 dimensions
Computed from entity_counters.completeness · refreshed after each connector run
Definition
A very rare, low-grade midline brain neoplasm that affects the septum pellucidum. It is characterized by similar histological features to those found in dysembryoplastic neuroepithelial tumor, but does not display multinodularity. It usually manifests with symptoms related to increased intracranial pressure. Epilepsy has been reported in approximately one third of patients. PDGFRA gene mutations have been identified in the majority of patients. Alterations in FGFR1 and NF1 genes have also been reported. BRAF mutations which represent the most common molecular alteration found in cortical dysembryoplastic neuroepithelial tumor, were absent in this rare group of midline tumors.
Data updated 23 days agoSource updated 26.08e
Taxonomy
Several hierarchies coexist; edges are listed per hierarchy type with their source.
Parents
Children (0)
Leaf node — no children.
Anatomy
Data updated 16 hours agoSource updated unknowncounters aggregate over descendants
Names
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Key figures
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No registry-level ancestor: burden figures are published for the mutually exclusive top-level site groups only, and this entity does not descend from one. Global figures (IARC / GLOBOCAN) stay under license review and SEER awaits credentials.
Data updated 16 hours agoSource updated unknownregistry figures: none · counters aggregate over descendants