We herein report an additional case of EWSR1-PATZ1 fusion-related round cell sarcoma in the face of a 5-year-old boy with unusual histologic features of pale zones, rosette/gland-like structures and expression of epithelial markers. Fluorescent in-situ hybridization study (FISH) using EWSR1 ...
Rakheja 2007 6 M L Hip focal/weak CD99+; diffuse FLI1+; EWS- primitive round cell sarcoma Lungs Rad Rx; complete surgical resection; vincristine, doxorubicin, cyclophosphamide; NED 7wks Yoshimoto 2009 14 M Head/Neck undifferentiated soft tissue sarcomas Lung DOD 9mo Yoshimoto 2009 16 F Trunk...
The present study describes a new case of EWSR1-negative undifferentiated sarcoma with CIC/ DUX4 gene fusion. This case is similar to tumors described as primitive undifferentiated round cell sarcomas that occur mainly in the trunk and display an aggressive behavior. To...
Abstract: Desmoplastic small round cell tumor (DSRCT) is an extremely rare, aggressive sarcoma affecting adolescents and young adults with male predominance. Generally, it originates from the serosal surface of the abdominal cavity. The hallmark characteristic of DSRCT is the EWSR1– WT1 gene ...
The new 5th edition of the WHO Classification of Tumors of Soft Tissue and Bone recognizes four subgroups of undifferentiated round cell sarcomas: Ewing sarcoma, CIC-rearranged sarcomas, BCOR-altered sarcomas, and sarcomas with EWSR1-non-ETS fusions, in addition to desmoplastic small round cell ...
round cell sarcoma with EWSR1‐non‐ETS fusionsundifferentiated small round cell sarcomasDue to the increased application of RNA‐based next‐generation sequencing techniques on bone and soft tissue round cell sarcomas new fusions are frequently found, thereby expanding the molecular landscape of these ...
sarcomaspindle and round cellneck tumorEWSR1㏄ATZ1 is a rare gene fusion recently recognized to occur in round and spindle cell sarcomas. To date, fewer than 20 cases have been described in the literature. However, no dedicated case reports have detailed its presentation in the head and neck...
The tumor's morphology and immunoprofile, along with molecular findings, led to a diagnosis of round cell sarcoma with EWSR1-CREM fusion. This case adds to the spectrum of tumors associated with this fusion, often presenting diverse morphologies. The rarity of EWSR1-...
Taken together, the histomorphologic and molecular findings were considered consistent with an undifferentiated round cell sarcoma with an EWSR1-CREB3L1 fusion. Although described in entities such as sclerosing epithelioid fibrosarcoma, low-grade fibromyxoid sarcoma, and small cell osteosarcoma, this has...
NFATc2-EWSR1, FUS, Ewing Sarcoma, Bone NeoplasmsBackground:The classification of bone neoplasms composed of small round cells is experiencing a transformation after the discovery of various gene fusion rearrangements that determine diagnosis, behavior, and response to therapy....