Intracranial Mesenchymal Tumor, FET-CREB Fusion-Positive

**Semantic type:** Neoplastic Process

**Definition:** A provisional entity that refers to a group of neoplasms 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.

**Synonyms:** - Intracranial Mesenchymal Tumor /Angiomatoid Fibrous Histiocytoma - Intracranial Mesenchymal Tumor with FET-CREB Fusion - Intracranial Mesenchymal Tumor, FET::CREB Fusion-Positive