NCIt 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.;