Ewing Sarcoma

We present a unique case of primary pelvic extraskeletal Ewing sarcoma in a previously healthy young woman with localized invasion into the uterus, vagina, and bladder. Given the rarity of this disease, paucity of data, and potential for misdiagnosis, clinicians must maintain high suspicion for pelvic extraskeletal Ewing sarcoma.

Key messages Fine‑needle aspiration appears to be at least as effective as core-needle biopsy for these malignancies. Cytomorphological features, together with the immunohistochemical profile and detection of CIC rearrangements, can help accurately diagnose this rare malignancy.

Primary renal Ewing sarcoma is an aggressive malignant tumor, often diagnosed at advanced stages with venous tumor thrombosis. The morphological heterogeneity and high rate of hemorrhage and necrosis are correlated with more invasive potential, suggesting prognostic significance.

This case highlights the diagnostic value of integrating NGS (DNA and RNA) and CMA in sarcomas with mixed or unusual histology. Such tools are essential for precise classification, prognostication, and consideration of targeted therapies in rare and histologically complex bone tumors.

Checkpoint inhibitors are also under evaluation, particularly in combination with immunomodulatory or targeted agents. Collectively, these trials highlight both the promise and limitations of immunotherapy in pediatric sarcomas and underscore the need for deeper understanding of sarcoma immune biology to guide future, more effective therapeutic strategies.

Systemic chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide was initiated, resulting in tumor shrinkage. To the best of our knowledge, this is the first reported case of BCS treated with CIRT. This case demonstrates that CIRT can achieve durable local control and suggests that it may represent a promising treatment option for BCS when surgical resection is challenging.

P1/2, N=20, Recruiting, Shenzhen Geno-Immune Medical Institute | Trial completion date: Dec 2023 --> Dec 2030 | Trial primary completion date: Nov 2023 --> Dec 2029

In conclusion, EWSAT1 promotes the malignant phenotypes of CRC cells by regulating the miR-330-5p/CPEB4 axis. This molecular axis may provide new insights into the mechanisms of CRC progression and potential targeted interventions.

CYP26A1 expression was most frequent in giant cell tumors of bone. While also detected in a subset of other bone tumors, expression was limited or absent in most benign and malignant lesions, and entirely absent in normal bone tissue. These findings provide novel descriptive insight into CYP26A1 distribution and support further investigation into its role in retinoid-related pathways in bone tumor biology.

P2, N=73, Recruiting, Northwestern University | N=29 --> 73 | Trial completion date: Jul 2029 --> Jul 2033

Consequently, the direct in vivo evidence for the role of NPY in metastasis and clinical data associating the expression of NPY and its receptor with adverse disease phenotypes are growing. Understanding the mechanisms underlying these effects may lead to the design of novel therapeutic approaches targeting the NPY system to prevent cancer progression.

The results of the screen revealed that a PI3K inhibitor, copanlisib, combined with a CDK4/6 inhibitor, ribociclib, exhibited strong synergistic anti-Ewing sarcoma activity. In two xenograft models of Ewing sarcoma, we demonstrated that the combination significantly prolonged survival compared to treatment with either vehicle or single-agent therapy alone. Our findings identify a new candidate therapy combination for Ewing sarcoma and provide a resource of additional potential synergistic combinations for future validation.