"EWSR1-CREB3L1 gene fusion or other rearrangements (e.g. FUS-CREB3L2 fusion)"
"EWSR1-CREB3L1 gene fusion or other rearrangements (e.g. FUS-CREB3L2 fusion)"
"Grossly sclerosing epithelioid fibrosarcomas are firm tumours of grey-white colour. They appear well-circumscribed on imaging, however, are histologically infiltrative. They range from 2-22cm in size, mean of 7-10cm. They usually involve the deep muscles and muscular fascia or adhere to the periosteum and occasionally might involve the bone 1-3. There might be calcifications 1."
"Grossly sclerosing epithelioid fibrosarcomas are firm tumours of grey-white colour. They appear well-circumscribed on imaging, however, are histologically infiltrative. They range from 2-22cm in size, mean of 7-10cm. They usually involve the deep muscles and muscular fascia or adhere to the periosteum and occasionally might involve the bone 1-3. There might be calcifications 1."
"Ober 60% of sclerosing epithelioid fibrosarcomas contain EWSR1-CREB3L1 gene fusions 1,3."
"T1: hypo- to isointense to muscle"
"T2: mixed-signal intensity with irregular hypointense areas"
"T1 C+ (Gd): heterogeneous and perilesional enhancement"
"EWSR1-CREB3L1 gene fusion or other rearrangements (e.g. FUS-CREB3L2 fusion)"
"Immunohistochemistry stains often express MUC4 and vimentin and may express EMA, CD99 or smooth muscle actin 1,3. They are usually negative for keratin 1,3."
"Grossly sclerosing epithelioid fibrosarcomas are firm tumours of grey-white colour. They appear well-circumscribed on imaging, however, are histologically infiltrative. They range from 2-22cm in size, mean of 7-10cm. They usually involve the deep muscles and muscular fascia or adhere to the periosteum and occasionally might involve the bone 1-3. There might be calcifications 1."
"Ober 60% of sclerosing epithelioid fibrosarcomas contain EWSR1-CREB3L1 gene fusions 1,3."
Expected headings
"Associations"
"Immunohistochemistry"
"Sclerosing epithelioid fibrosarcomas are rare aggressive mesenchymal malignancies characterised by a dense sclerotic hyalinized stroma with cords, nests and areas of epithelioid fibroblasts."
"The diagnosis of sclerosing epithelioid fibrosarcoma is established by histological, immunohistochemical and molecular genetic criteria 1."
"epithelioid cells in a dense sclerotic collagenous background, organised in nests, cords or sheets"
"nests, cords or sheets of uniform ovoid epithelioid cells with scant clear to eosinophilic cytoplasm in a hyalinized sclerotic extracellular matrix"
"Immunohistochemistry stains often express MUC4 and vimentin and may express EMA, CD99 or smooth muscle actin 1,3. They are usually negative for keratin 1,3."
"form, location and size"
"This malignancy takes an aggressive clinical course with high mortality Local recurrence and metastases are common and occur in about 50% of cases. The most common sites for metastasis are the lung, pleura, bone and brain 1-4. Oligometastatic disease to the lungs may be treated with surgical resection, which improves prognosis. The tumours do not respond to conventional chemotherapy, although there are current clinical trials in immunotherapy, although data is limited due to the rarity of this malignancy."
"EWSR1-CREB3L1 gene fusion or other rearrangements (e.g. FUS-CREB3L2 fusion)"