intracranial mesenchymal tumor, FET-CREB fusion-positive
Findings
No curated finding names intracranial mesenchymal tumor, FET-CREB fusion-positive yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A central nervous system mesenchymal non-meningothelial tumor with a broad morphological spectrum, characterized by fusion of a FET family gene (usually EWSR1 and rarely FUS) with a member of the CREB family of transcription factors (CREB1, ATF1, or CREM). It includes entities previously termed intracranial angiomatoid fibrous histiocytoma or intracranial myxoid mesenchymal tumor. It is usually located in the supratentorial brain and mostly affects children and young adults. There is a spectrum of clinical behaviors ranging from slowly growing tumors to rapid recurrences, and rarely metastases.
Definition from the Mondo Disease Ontology (MONDO:1060252), read 2026-09-29. CC BY 4.0.
Where it sits
Other names
2 names
Resolves to: intracranial mesenchymal tumor, FET-CREB fusion-positive
- Also called
- intracranial mesenchymal tumor /angiomatoid fibrous histiocytomaintracranial mesenchymal tumor with FET-CREB fusion