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Background: A subset of undifferentiated small round cell sarcomas (USRCSs) is currently being recognized as emerging entities with unique gene fusions: CIC-DUX4 (the area of focus in this article), BCOR-CCNB3, or CIC-FOXO4 gene fusions. CIC-DUX4 and CIC-FOXO4 fusions have been reported in soft tissue tumors, while BCOR-CCNB3 fusion with an X chromosomal inversion was described in both bone and soft tissue tumors. CIC-DUX4 fusion can either harbor t(4;19)(q35;q13.1) or t(10;19)(q26.3;q13), while t(4;19)(q35;q13.1) is reported more commonly., Case Report: The aim of this study is to share a new case report of a 36-year-old woman who had a rapidly growing mass in her right upper thigh, which was found to be an undifferentiated small round cell sarcoma with t(4;19)(q35;q13.1) CIC-DUX4 fusion was confirmed by cytogenetic testing. Combined modality treatment with surgery, radiation, and chemotherapy was used and achieved a good response. A review of the literature of the reported cases with CIC-DUX4 fusions including both t(4;19) and t(10;19) translocations revealed a total of 44 cases reported. Out of these 44 cases, 33 showed t(4;19)(q35;q13.1) translocation compared to 11 cases with t(10;19)(q26.3;q13)., Conclusions: Undifferentiated small round cell sarcomas are aggressive tumors. Their treatment includes surgery, chemotherapy, and radiation. Resistance to chemotherapy is common. Lung and brain are common sites of metastasis, with associated poor prognosis. Generally, median survival is less than 2 years. Newer techniques have been developed recently which helped identify a subset of previously unclassifiable sarcomas, with promising prognostic value.

Acetone-fixed cryostat sections of 79 breast carcinomas were immunostained with antibodies to basic fibroblast growth factor (bFGF) and urokinase-type plasminogen activator (uPA). Staining intensity was then compared with microvessel density assessed by manually counting vascular spaces highlighted by immunostaining vascular basal lamina (Type IV) collagen. Extensive (2+) bFGF immunoreactivity was present in neoplastic cells of 30 tumors (38%) and in host-derived stromal cells of 29 cases (37%). Disease recurrence correlated with bFGF staining: 0 to 1+ stromal staining, 30% recurred versus 2+ stromal staining, 73% recurred (P = 0.001) (54-mo median follow-up). Neither stromal nor epithelial bFGF staining correlated significantly with microvessel count; however, there was a statistically significant association between stromal cell bFGF staining and uPA staining of peritumor host cells: absent bFGF--0% 2+ uPA versus weak bFGF--9% 2+ uPA versus 2+ bFGF--29% uPA (P = 0.01). We conclude that elevated expression of bFGF in breast carcinomas is associated with aggressive clinical behavior. Its biologic significance, however, appears more closely related to extracellular matrix remodeling than to induction of prominent neovascularization per se.