Sclerosing epithelioid fibrosarcoma (SEF) is a rare and distinct deep-seated fibrosarcoma that often affects adults. It was first identified in 1995, and the presence of it was later verified by others. Nests and cords of uniform small epithelioid cells embedded in a tightly sclerotic matrix are classic histologic characteristics of SEF. Its fibroblastic form, complete with a thick collagenous matrix indistinguishable from osteoid, has been proved through ultrastructural studies. SEF may have minor fibroma or low grade fibromyxoid sarcoma (LGFMS)-like zones, cysts, and calcification, but these is usually minor. Recent research has highlighted the morphologic, immunohistochemical, and molecular similarities between SEF and LGFMS, leading researchers to believe that the two tumours are closely related. Evans was the first to recognise LGFMS as a distinct entity, with bland, whorled fibrous areas with myxoid zones and thin-walled blood vessels. The morphologic spectrum of LGFMS includes tumours previously classified as "hyalinizing spindle cell tumour with giant rosettes (HSCTGR). MUC4, a high-molecular-weight transmembrane glycoprotein, is a sensitive immunohistochemical marker that shows good cytoplasmic expression in both LGFMS (99% to 100%) and SEF (at least 78 percent). LGFMS has historically demonstrated either the translocation resulting in FUS-CREB3L2 fusion (96%) or the translocation resulting in FUS-CREB3L1 fusion (96%) on a molecular basis (4 percent). Epithelioid sarcoma is a rare soft tissue sarcoma that develops in mesenchymal tissue and has epithelioid-like characteristics. It only accounts for around 1% of all soft tissue sarcomas. It usually appears as a thin, soft mass or a series of bumps in the distal limbs (fingers, shoulders, forearms, or feet) of young adults. There is also a proximal variant, which is more common in the upper extremities. The pelvis, vulva, penis, and spine have all been confirmed to have rare cases. Inactivation of the SMARCB1 gene, or loss of INI-1 activity, is the most common genetic mutation (found in 80-90 percent of epithelioid sarcomas), and it is thought to be a major contributor to disease progression. Tissue biopsy is the preferred method of diagnosis. A sentinel lymph node biopsy is often performed due to the high incidence of lymph node involvement. The loss of function of the SMARCB1 gene is a typical feature of epithelioid sarcoma (it occurs in 80% of cases) (also termed BAF47, INI1, or hSNF5). Those who are interested to publish their article in our journal, they can submit it either send it as an email attachment to this below given mail id or submit it online through given link: https://www.longdom.org/submissions/medical-surgical-pathology.html

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ISSN: 2472-4971 | NLM ID: 101245791