"background stroma varies from mucin-rich to collagenous; can sometimes be nearly absent."
"dense lymphoplasmacytic cuffing peripherally or along fibrous septa with germinal centre formation."
"SWI: foci of hypo intensity are often peripheral and reflect intratumoural haemorrhagic changes 4."
"These tumours are characterised by the fusion of a FET RNA-binding protein family gene, which includes Ewing sarcoma RNA binding protein 1 (EWSR1) and fused in sarcoma (FUS), to a cAMP response element-binding protein (CREB) family gene, which includes activating transcriptase factor-1 (ATF1), cAMP responsive element binding protein 1 (CREB1), cAMP response element modulator (CREM), and cAMP responsive element binding protein 3 like 3 (CREB3L3) 1-5."
"DWI/ADC: does not typically restrict diffusion"
"Intracranial mesenchymal tumours, FET-CREB fusion-positive, are rare only recently described soft tissue neoplasms of intermediate malignancy. They are characterised by the fusion of the FET family of RNA-binding proteins to the CREB family of transcription factors, also seen in extracranial angiomatoid fibrous histiocytoma 1."
"Intracranial mesenchymal tumours, FET-CREB fusion-positive, are rare only recently described soft tissue neoplasms of intermediate malignancy. They are characterised by the fusion of the FET family of RNA-binding proteins to the CREB family of transcription factors, also seen in extracranial angiomatoid fibrous histiocytoma 1."
"Intracranial mesenchymal tumours, FET-CREB fusion-positive, are rare only recently described soft tissue neoplasms of intermediate malignancy. They are characterised by the fusion of the FET family of RNA-binding proteins to the CREB family of transcription factors, also seen in extracranial angiomatoid fibrous histiocytoma 1."
"Intracranial mesenchymal tumour, FET-CREB fusion-positive have been added to the 5th Edition of the WHO classification of CNS tumours as a provisional entity and as yet have been assigned a grade 5."
"The specific features present also vary according to the type of FET-CREB mutation involved 1."
"EMA: frequently positive"
"These tumours are characterised by the fusion of a FET RNA-binding protein family gene, which includes Ewing sarcoma RNA binding protein 1 (EWSR1) and fused in sarcoma (FUS), to a cAMP response element-binding protein (CREB) family gene, which includes activating transcriptase factor-1 (ATF1), cAMP responsive element binding protein 1 (CREB1), cAMP response element modulator (CREM), and cAMP responsive element binding protein 3 like 3 (CREB3L3) 1-5."
"These tumours are characterised by the fusion of a FET RNA-binding protein family gene, which includes Ewing sarcoma RNA binding protein 1 (EWSR1) and fused in sarcoma (FUS), to a cAMP response element-binding protein (CREB) family gene, which includes activating transcriptase factor-1 (ATF1), cAMP responsive element binding protein 1 (CREB1), cAMP response element modulator (CREM), and cAMP responsive element binding protein 3 like 3 (CREB3L3) 1-5."
"These tumours are characterised by the fusion of a FET RNA-binding protein family gene, which includes Ewing sarcoma RNA binding protein 1 (EWSR1) and fused in sarcoma (FUS), to a cAMP response element-binding protein (CREB) family gene, which includes activating transcriptase factor-1 (ATF1), cAMP responsive element binding protein 1 (CREB1), cAMP response element modulator (CREM), and cAMP responsive element binding protein 3 like 3 (CREB3L3) 1-5."
"Intracranial mesenchymal tumours, FET-CREB fusion-positive, are lobulated extra-axial, or less frequently intraventricular, tumours with dural attachments 1,2. They often have solid and cystic components 1,2,5. They are located anywhere in the neuraxis (including in the spine) 1,2."
"The primary imaging differential diagnoses are entities which also present as extra-axial or intraventricular tumours. Taking into account the rarity of intracranial mesenchymal tumours, FET-CREB fusion-positive, and their variable imaging appearance they are usually diagnosed on the basis of histology, and no reliable imaging features allow for their pre-operative diagnosis."
"These tumours have been previously referred to as intracranial myxoid mesenchymal tumour or intracranial angiomatoid fibrous histiocytoma 1."
"These tumours have been previously referred to as intracranial myxoid mesenchymal tumour or intracranial angiomatoid fibrous histiocytoma 1."
"Histologically, these tumours are heterogenous with variable morphology 1,5."
"heterogenous moderate to intense enhancement"
"EWSR1-CREB1 fusion: mucin-rich stroma, with a more stellate or spindle cell morphology, and haemangioma-like vasculature"
"EWSR1-ATF1 fusions: sheets of epithelioid cells with mucin-poor, collagenous stroma"
"These tumours are characterised by the fusion of a FET RNA-binding protein family gene, which includes Ewing sarcoma RNA binding protein 1 (EWSR1) and fused in sarcoma (FUS), to a cAMP response element-binding protein (CREB) family gene, which includes activating transcriptase factor-1 (ATF1), cAMP responsive element binding protein 1 (CREB1), cAMP response element modulator (CREM), and cAMP responsive element binding protein 3 like 3 (CREB3L3) 1-5."
Expected headings
"Terminology"
"Intracranial mesenchymal tumours, FET-CREB fusion-positive, are rare only recently described soft tissue neoplasms of intermediate malignancy. They are characterised by the fusion of the FET family of RNA-binding proteins to the CREB family of transcription factors, also seen in extracranial angiomatoid fibrous histiocytoma 1."
"Patients typically present with symptoms caused by the mass effect of the tumour, such as headaches, nausea, tinnitus, diplopia, seizures, and focal neurological deficits. Fever of unknown origin has also been reported in IMT, associated with tumour-derived Interleukin-6 2."
"These tumours are characterised by the fusion of a FET RNA-binding protein family gene, which includes Ewing sarcoma RNA binding protein 1 (EWSR1) and fused in sarcoma (FUS), to a cAMP response element-binding protein (CREB) family gene, which includes activating transcriptase factor-1 (ATF1), cAMP responsive element binding protein 1 (CREB1), cAMP response element modulator (CREM), and cAMP responsive element binding protein 3 like 3 (CREB3L3) 1-5."
"Although similar genetic abnormalities are identified in extracranial angiomatoid fibrous histiocytoma, it remains unclear whether or not these should be considered the same entity, especially as similar gene fusions are encountered in other soft tissue tumours (e.g. clear cell sarcoma of soft tissue, gastrointestinal clear cell sarcoma, hyalinizing clear cell carcinoma of the salivary gland, primary pulmonary myxoid sarcoma) 1,5."
"background stroma varies from mucin-rich to collagenous; can sometimes be nearly absent."
"These tumours are characterised by the fusion of a FET RNA-binding protein family gene, which includes Ewing sarcoma RNA binding protein 1 (EWSR1) and fused in sarcoma (FUS), to a cAMP response element-binding protein (CREB) family gene, which includes activating transcriptase factor-1 (ATF1), cAMP responsive element binding protein 1 (CREB1), cAMP response element modulator (CREM), and cAMP responsive element binding protein 3 like 3 (CREB3L3) 1-5."